KIT CARDIACO OBSTÉTRICO APROXIMACIÓN PRÁCTICA BASADA EN ETAPAS Jorge Rubio. Anestesiólogo Cardiovascular. UAD. Clínica del Prado. Medellín. Germán Monsalve. Anestesiólogo. Director UAD. Clínica del Prado. Medellín. Martín Gómez. Anestesiólogo. Profesor Universidad CES. HUSVP. Medellín. Mauricio Vasco. Anestesiólogo. ME. Profesor Unisanitas. Jefe Anestesia Obstétrica. Clínicas Colsanitas. Bogotá. La enfermedad cardíaca es la primera causa de muerte materna en el Reino Unido, representando un mayor número de casos que la hemorragia y los trastornos hipertensivos juntos (Enfermedad Cardíaca n: 48, Hemorragia n: 14, Trastornos Hipertensivos n: 18). Yentis revisó las muertes maternas debidas a enfermedad cardíaca en los últimos treinta años en el Reino Unido encontrando que la enfermedad cardíaca congénita representa el 26 % del total, siendo la primera causa. El pobre cuidado preconcepcional y no conocer el diagnóstico de la enfermedad antes de embarazarse, unido a un cuidado subóptimo, principalmente en el postparto, son las principales causas para un mal resultado. Aunque el 0.8 % de la población general tiene una enfermedad cardíaca congénita, hasta un 85 % llegan a ser adultos, por lo que se considera que esta población está en aumento en el mundo. Un manejo multidisciplinario y enfocado por etapas (Preconcepción, Prenatal, Intraparto y Postparto) es propuesto para una aproximación práctica de la paciente obstétrica con enfermedad cardíaca.

MANEJO POR ETAPAS ● PRECONCEPCIONAL ÓPTIMA CONSEJERIA I. CLASIFICACIÓN DEL RIESGO 1. RIESGO MATERNO A. ANATOMO – FISIOPATOLÓGICA B. CARPREG ➔ Probabilidad de evento cardíaco mayor ➢ Primario ➢ Secundario FACTORES DE RIESGO ➔ Probabilidad de evento obstétrico FACTORES DE RIESGO C. CIRCULATION ➔ Probabilidad de evento cardíaco mayor ➢ Primario ➢ Secundario FACTORES DE RIESGO ➔ Probabilidad de evento obstétrico FACTORES DE RIESGO D. JACC

➔ ➢ ➢ ➔

Probabilidad de evento cardíaco mayor Primario Secundario FACTORES DE RIESGO Probabilidad de evento obstétrico FACTORES DE RIESGO

E. ENFERMEDAD VALVULAR GUIAS ACC/AHA ➔ Severidad ➔ Indicaciones valvulotomia percutánea 1. ESTENOSIS AÓRTICA 2. INSUFICIENCIA AÓRTICA 3. ESTENOSIS MITRAL 4. INSUFICIENCIA MITRAL 5. ESTENOSIS PULMONAR 6. INSUFICIENCIA PULMONAR 7. ESTENOSIS TRICUSPÍDEA 8. INSUFICIENCIA TRICUSPÍDEA F. CARDIOMIOPATIA PERIPARTO CRTERIOS DIAGNÓSTICOS FACTORES DE RIESGO FACTORES PRONÓSTICOS 2. RIESGO FETAL A. CARPREG ➔ Probabilidad de eventos FACTORES DE RIESGO B. CIRCULATION ➔ Probabilidad de eventos FACTORES DE RIESGO C. JACC ➔ Probabilidad de eventos FACTORES DE RIESGO 3. RIESGO DE RECURRENCIA 4. CONSEJERIA II. PLANIFICACIÓN III. OPTIMIZACIÓN 1. LESIONES ESTENÓTICAS 2. LESIONES REGURGITANTES 3. CARDIOMIOPATIA PERIPARTO 4. DISFUNCIÓN VENTRICULAR

● PRENATAL

MANEJO MULTIDISCIPLINARIO I.MANEJO TEMPRANO III NIVEL II.TERMINACIÓN DEL EMBARAZO III.ANTICOAGULACIÓN 1. CLASIFICACIÓN DEL RIESGO TROMBOEMBÒLICO MATERNO A. Prótesis mecánica B. Tromboembolismo venosos FACTORES DE RIESGO C. Anticuerpos antifosfolípidos APLAS FACTORES DE RIESGO 2. PRIORIDAD DE SEGURIDAD MATERNA vs FETAL ➔

PROTESIS VALVULAR MECÁNICA



Riesgo Trombótico Alto



Riesgo Trombótico “Relativamente Bajo

➔

TEV

➔

APLAs

➔

PREECLAMPSIA 3. TERAPIA PUENTE A. HEPARINA NO FRACCIONADA (HNF) B. HEPARINA BAJO PESO MOLECULAR (HBPM)

IV.DROGAS CARDÍACAS 1. “RELATIVAMENTE” SEGURAS 2. NO SEGURAS V.PLAN – LECTURA CRÍTICA VI.CIRUGÍA CARDÍACA 1. CLASIFICACIÓN DEL RIESGO

A. RIESGO MATERNO FACTORES DE RIESGO B. RIESGO FETAL FACTORES DE RIESGO 2. PROTECCIÓN FETAL - BCP

• INTRAPARTO APROXIMACIÓN CON EL MÍNIMO I. PROBLEMAS 1. HEMORRAGIA 2. OXITOCINA – CARBETOCINA 3. EDEMA PULMONAR 4. ARRITMIAS 5. DISMINUCIÓN RVS 6. HIPERTENSIÓN PULMONAR 7. TROMBOEMBOLISMO 8. ENDOCARDITIS BACTERIANA II.MANEJO DEL PARTO 1. MONITOREO ASA Vs Invasivo 2. ANALGESIA Epidural Vs CSE 3. VÍA DEL PARTO Vaginal Vs Cesárea 4. TÉCNICA ANESTÉSICA II. A. General Vs Regional         

RIESGO DE COMPLICACIONES DISMINUCIÓN RVS – INOTROPISMO HIPERTENSIÓN PULMONAR (HTP) RIESGO TROMBOEMBÓLICO RIESGO DE MUERTE MATERNA VENTILACIÓN MECÁNICA POSTPARTO CIRUGÍA PROLONGADA PREDICTORES VÍA AÉREA DIFÍCIL PREFERENCIA DEL ANESTESIÒLOGO B. Obstétrica Vs Cardíaca PATOLOGÍAS ESTENÓTICAS

RIESGO

    

Resistencias vasculares sistémicas (RVS) Precarga – Postcarga Catecolaminas Frecuencia Vía aérea

● POSTPARTO EVITAR CUIDADO SUBÓPTIMO I. MONITOREO 1. SITIO A. UCI B. UCE C. UAD 2. TIEMPO II.

PLANIFICACIÓN

CONCLUSIONES        ● ● ●

ENFERMEDAD CARDÍACA: MUERTE MATERNA CARDIOPATÍA CONGÉNITA EN AUMENTO HTP – SÍNDROME DE EINSENMEGER…. OJO DIAGNÓSTICO DURANTE EL EMBARAZO LECTURA CRÍTICA DE PARACLÍNICOS ENFOQUE POR SECCIONES PRECONCEPCIONAL: ÓPTIMA CONSEJERÍA PRENATAL: MANEJO MULTIDISCIPLINÁRIO INTRAPARTO: APROXIMACIÓN CON EL MÍNIMO RIESGO POSTPARTO: EVITAR CUIDADO SUBÓPTIMO

● PRECONCEPCIONAL I. CLASIFICACIÓN DEL RIESGO 1. RIESGO MATERNO¹ ALIDAD 0.1% - 1.0%)1% - 1.0%) A. ANATÓMICO – FISIOPATOLÓGICA



BAJO RIESGO

( 0,1% - 1% )

Cortocircuitos izquierda – derecha no complicados • Prolapso Mitral – Válvula Aórtica bicúspide Insuficiencia Aórtica – Insuficiencia Mitral – Estenosis pulmonar – Insuficiencia pulmonar • Lesiones corregidas •

 RIESGO INTERMEDIO

( 1% - 5% )

•

Prótesis valvulares mecánicas Ventrículos únicos – Ventrículo derecho sistémico – Cx. Suiche Lesiones cianóticas no corregidas Estenosis Mitral – Estenosis Aórtica leve/moderada – Estenosis pulmonar severa Tetralogía de Fallot



RIESGO ALTO

• • • •

• • • • •

( 5% - 30% )

NYHA III – IV Disfunción ventricular severa Hipertensión pulmonar severa Estenosis Aórtica severa Síndrome Marfan: Lesión valvular – Dilatación Aórtica B. ENFERMEDAD CARDÍACA CARPREG²

➔

Probabilidad de Evento Cardíaco Mayor

➢

Primario Edema pulmonar – Falla cardíaca Arritmia sintomática sostenida Muerte cardíaca Paro cardíaco Evento cerebro-vascular

    

0 = 5%

1 = 27%

13%

2 = 75%

Secundario o Arritmia sintomática no sostenida o Deterioro NYHA ≥ 2 o Procedimiento cardíaco invasivo hasta 6m post-parto Incidencia 6% ➢

FACTORES DE RIESGO     • • •

Evento cardíaco – Arritmia Clase NYHA III o IV – Cianose o SatO2 < 90% Disfunción ventricular FE < 40% Obstrucción Corazón izquierdo Área valvular mitral Área valvular Aórtica Gradiente máximo tracto de salida VI

< 2cm² < 1,5cm² > 30mm

Probabilidad de Evento Obstétrico o Muerte no cardíaca o Trastorno hipertensivo asociado al embarazo (THAE) o Hemorragia post parto Parto vaginal > 500mls Cesárea > 1000mls Transfusión ↓ Hb > 2gr/dl ➔

0,3% 4% 3%

C. ENFERMEDAD CARDÍACA CONGÉNITA CIRCULATION³ ➔

Probabilidad de Evento Cardíaco Mayor

Primario 0 = 12% 1 = 30% ➢ Secundario Incidencia 12,5% FACTORES DE RIESGO

19,4%

➢

 ● ● ●  ➔

o o o

o

2 = 100%

Evento cardíaco – Arritmia Clase NYHA III o IV – Cianose o SatO2 < 90% Disfunción ventricular subpulmonar FE < 40% Insuficiencia pulmonar severa Tabaquismo Probabilidad de Evento Obstétrico 10,6% Muerte no cardíaca 0 Trastorno hipertensivo asociado al embarazo (THAE) 2,8% Hemorragia post parto 7,8% Parto vaginal > 500mls Cesárea > 1000mls Transfusión ↓ Hb > 2gr/dl Abortos 20% Espontáneos 12,2% Electivos 7,8% D. ENFERMEDAD CARDÍACA CONGÉNITA JACC4

➔

o o o o

Probabilidad de Evento Cardíaco Mayor Falla cardíaca Arritmia sintomática sostenida Eventos cardiovasculares IAM – Muerte cardíaca – ACV Endocarditis

11% 4,8% 4,5% 1,9% 0,5%

FACTORES DE RIESGO Cardiopatía congénita compleja: Cianosante – Síndrome de Einsenmeger – CIV + Atresia pulmonar  Mortalidad: Síndrome de Einsenmeger 



Endocarditis: CIA

➔

Probabilidad de Evento Obstétrico FACTORES DE RIESGO

34,2%

 Preeclampsia: Estenosis Aórtica – Estenosis pulmonar – Coartación Aórtica – Transposición de grandes arterias o Trastorno hipertensivo asociado al embarazo (THAE) 5,5% o Preeclampsia 3,2% o Ruptura prematura de membranas 3,9% o Parto prematuro 11% o Hemorragia post parto 8,4% o Eventos tromboembólicos 2,2% o Abortos 20% Espontáneos 15% Electivos 5% E. ENFERMEDAD CARDÍACA VALVULAR RIESGO MATERNO FETAL 5 GUIAS ACC/AHA. 2008 FACTORES DE RIESGO 1. Estenosis Aórtica severa 2. Insuficiencia Aórtica y clase funcional NYHA III–IV 3. Estenosis Mitral y clase funcional NYHA II–IV 4. Insuficiencia Mitral y clase funcional NYHA III–IV 5. Enfermedad valvular Aórtica o Mitral con hipertensión pulmonar severa (Presión pulmonar > 75% de la PA sistémica – PSAP > 50mm Hg) 6. Enfermedad valvular Aórtica o Mitral y disfunción VI severa (FE < 0.40) 7. Prótesis valvular mecánica y anticoagulación 8. Síndrome de Marfan 1. ESTENOSIS AÓRTICA  o

(EA)

SEVERA – RIESGO ALTO

Área valvular o Gradiente medio transvalvular o Velocidad del jet o Fracción de eyección

< 1.0 cm2 > 50mm Hg > 4mt/s < 45%

VALVULOTOMIA AORTICA PERCUTÁNEA Clase IIb 1. Terapia puente en pacientes adultos inestables, con riesgo alto para cambio valvular Aórtico. (Nivel de Evidencia: C) 2. Procedimiento paliativo en pacientes adultos, con enfermedades coexistentes severas, no candidatos para el cambio valvular Aórtico. (Nivel de Evidencia: C) o Class III o

No se recomienda como alternativa al cambio valvular Aórtico en los pacientes adultos; algunos adultos jóvenes sin calcificación pueden ser una excepción. (Nivel de Evidencia: B) o EMBARAZO 1. EA sintomática 2. INSUFICIENCIA AÓRTICA

(IA)

 SEVERA – RIESGO ALTO  CUALITATIVA o Clasificación angiográfica o Jet o Vena contracta  o o o o

CUANTITATIVA Volumen regurgitante Fracción regurgitante Área de orificio regurgitante Tamaño VI

3. ESTENOSIS MITRAL

3-4+ Central - > 65% Tracto de salida VI ≥ 0,7cm ≥ 60mls/lat ≥ 50% ≥ 0,3cm² Aumentado (EM)

 SEVERA – RIESGO ALTO Área valvular < 1.0 cm2 o Gradiente transvalvular > 10mm Hg o Presión sistólica arteria pulmonar (PSAP) > 50mm Hg o

VALVULOTOMIA MITRAL PERCUTÁNEA Requisito: Anatomía favorable – Trombo auricular izquierdo (AI) negativo - IM moderada o severa negativa o Clase I 1. EM moderada o severa y Clase funcional ≥ II. (Nivel de Evidencia A) 2. EM moderada o severa asintomática y PSAP > 50mm Hg reposo > 60mm Hg ejercicio. (Nivel de Evidencia C) o Clase IIa 1. EM moderada o severa y Clase funcional III – IV y riesgo alto o no candidatos para el cambio valvular Mitral. (Nivel de Evidencia C) o Clase IIb 1. EM moderada o severa asintomática y fibrilación auricular nueva. (Nivel de evidencia C) 2. EM moderada o severa y Clase funcional ≥ II y Área > 1,5cm² si: a. PASP > 60mm Hg b. PCCP > 25mm Hg c. Gradiente medio transvalvular > 15mm Hg

3. Terapia alternativa al cambio valvular Mitral en EM moderada o severa y Clase funcional III – IV. (Nivel de Evidencia C) o Clase III 1. No indicada en EM leve. (Nivel de Evidencia C) 2. No indicada en EM moderada y severa y trombo AI. (Nivel de Evidencia C) o EMBARAZO 1. EM sintomática preconcepcional 2. EM y Clase funcional III – IV durante el embarazo 3. EM y manejo médico óptimo fallido 4. INSUFICIENCIA MITRAL 

(IM)

SEVERA – RIESGO ALTO

 CUALITATIVA

o Clasificación angiográfica o Jet o Vena contracta

3-4+ Central - > 40% Área AI ≥ 0,7cm

CUANTITATIVA Volumen regurgitante Fracción regurgitante Área de orificio regurgitante o Tamaño VI o Tamaño AI

≥ 60mls/lat ≥ 50% ≥ 0,4cm² Aumentado Aumentado

 o o o

5. ESTENOSIS PULMONAR 

SEVERA

o Velocidad del jet o Gradiente transvalvular máximo

> 4mt/s >60mm Hg

6. INSUFICIENCIA PULMONAR 

SEVERA

o

Jet Desaceleración onda Doppler

o

Central Tracto de salida VD Lenta y continua

7. ESTENOSIS TRICUSPÍDEA 

SEVERA

o

Área valvular

8. INSUFICIENCIA TRICUSPÍDEA 

SEVERA

< 1.0 cm2

o o

Vena contracta ≥ 0,7cm Flujo sistólico reverso en venas hepáticas

F. CARDIOMIOPATÍA PERIPARTO 6-16 

FACTORES DE RIESGO

o o o o o o

Multiparidad Embarazo múltiple Preeclampsia Edad > 35 años Raza afro-americana Terapia tocolítica prolongada ?



CRITERIOS DIAGNÓSTICOS

o ICC 9º - 5 m Postparto o Descartar otras etiologías o Disminución de la Función VI 1. FE < 40% 2. FA < 30% 3. Diámetro DFVI > 2,7cm/m² Biopsia endomiocárdica: Síntomas persistentes > 2 semana 

ETIOLOGÍA

o Infecciosa: Miocarditis → Virus Coxsakie – Eco virus → Clamidia pneumonie o Inmunológica: Citokinas inflamatorias aumentadas (FNTα-IL6-Fas/Apo-1) → Anticuerpos contra la actina → Anticuerpos contra proteínas musculares cardíacas o Nutricional → Dieta hipersódica → Deficiencia de selenio? o Farmacológica → Terapia tocolítica prolongada? o Familiar 

CRITERIOS PRONÓSTICOS

o → → → → → → → →

EVALUACIÓN INICIAL FE VI basal < 25% - 6 meses FA < 20% Diámetro DFVI > 6cm/m² Presentación clínica > 2 sems posparto Edad > 30 años Raza afro-americana Multiparidad Fas/Apo-1 (receptor de apoptosis) elevado

o PRUEBA STRESS DOBUTAMINA → FE normal + Reserva contráctil disminuida o → → →

IMPLICACIONES Peor evolución clínica Trasplante cardiaco Consejería NO embarazo 2. RIESGO FETAL B. ENFERMEDAD CARDÍACA CARPREG²

➔      

Probabilidad de Evento Fetal Parto prematuro < 37sems Bajo peso para EG < Percentil 10 SDR HIV Muerte fetal Muerte neonatal

20% 15,2% 2,8% 5% 5% 1% 1%

FACTORES DE RIESGO     

Clase NYHA III o IV – Cianose o SatO2 < 90% Obstrucción Corazón izquierdo Anticoagulación Tabaquismo Gestación múltiple

0 = 2%

1 = 4%

2 = 100%

B. ENFERMEDAD CARDÍACA CONGÉNITA CIRCULATION³ ➔      

Probabilidad de Evento Fetal Parto prematuro < 37sems Bajo peso para EG < Percentil 10 SDR HIV Muerte fetal Muerte neonatal

27,8% 20,8% 8,3% 8,3% 1,4% 2,8% 1,4%

FACTORES DE RIESGO •

Gradiente máximo tracto de salida VI

> 30mm

C. ENFERMEDAD CARDÍACA CONGÉNITA JACC 4

➔       

Probabilidad de Evento Fetal Parto prematuro < 37sems Bajo peso para EG < Percentil 10 SDR HIV Muerte fetal Muerte neonatal Transmisión Enfermedad cardíaca congénita

15,9% 8% 8,3% 1,4% 1,7% 2,3% 3,5%

FACTORES DE RIESGO Cardiopatía congénita cardíaca: Cianosante – Síndrome de Einsenmeger – CIV + Atresia pulmonar – Transposición de grandes arterias – Fontan 

3. ENFERMEDAD CARDÍACA CONGÉNITA RIESGO TRANSMISIÓN ¹ 9-13 o Luminiscencia nucal 12 – 13 sems (1/1000 si es normal) o Ecocardiografía fetal 14 – 16 sems y 18 – 22 sems       

Tetralogía de Fallot Ductus Arterioso Persistente – Coartación de Aorta CIA Estenosis pulmonar CIV – Comunicación AV Estenosis Aórtica Síndrome Marfan – Síndrome de Di George

2–3% 4% 5 – 11% 6–7% 10 – 16% 15 – 18% 50%

4. ENFERMEDAD CARDÍACA CONSEJERIA 14-18 A. ● ● ● ● ●

B. C. D. E.

No embarazo Síndrome de Einsenmeger severo Cardiopatía congénita cianosante severa Hipertensión pulmonar severa Síndrome de Marfan Aorta > 4cms Cardiomiopatía periparto con factores de mal pronósticos Expectativa materna de vida Nivel de seguimiento Nivel de tratamiento Hospitalización anticipada en el embarazo

II. PLANIFICACIÓN ¹ 19-20 1. ANTICONCEPTIVOS ORALES COMBINADOS - CONTRAINDICADOS (RIESGO TROMBOSIS) 2. PROGESTERONA SOLA ORAL (CERAZETTE) – SEGURO Y EFECTIVO 3. PROGESTERONA SOLA IMPLANTE (IMPLANON) – SEGURO Y EFECTIVO

4. DISPOSITIVO INTRAUTERINO – PROFILAXIS Y REACCIÓN VASOVAGAL 5. TUBECTOMÍA – EMBOLISMO Y NEUMOPERITONEO III. OPTIMIZACIÓN 5 - 21 1. LESIONES ESTENÓTICAS 

MEDIDAS GENERALES

o Restricción hídrica (< 2Lts/día) o Restricción de sal (< 4grs/día) o Ejercicio físico moderado 

MEDIDAS FARMACOLÓGICAS

o ORALES  → → → →

PREPARTO Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos? α Bloqueadores

 → → → → →

POSTPARTO IECA? – ARA? Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos? α Bloqueadores Bloqueadores

→

o INTRAVENOSAS Síntomas severos – No respuesta a tratamiento VO → Dopamina 0,25 – 5 µgrs/kg/min → Nitratos 1 – 10 mg/h → Dobutamina 2 – 20 µgrs/kg/min → Milrinone Bolo: 25 – 75 µgrs/kg en 10 min Infusión: 0,25 – 1 µgrs/kg/min → Nitroprusiato de sodio 0,3 – 5 µgrs/kg/min → Levosimendan Bolo: 12 µgrs/kg en 10 min Infusión: 0,05 – 0,2 µgrs/kg/min o ANTICOAGULACIÓN: → FA > 48 horas → AP: Embolismo → FA crónica → AI > 55mm o VALVULOPLASTÍA PERCUTÁNEA DE RESCATE 2. LESIONES REGURGITANTES

 o o o

MEDIDAS GENERALES Restricción hídrica (< 2Lts/día) Restricción de sal (< 4grs/día) Ejercicio físico moderado



MEDIDAS FARMACOLÓGICAS

o ORALES  → → → →

PREPARTO Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos α Bloqueadores

 → → → → →

POSTPARTO IECA – ARA Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos α Bloqueadores Β Bloqueadores

→

o INTRAVENOSAS Síntomas severos – No respuesta a tratamiento VO → → → →

Dopamina Nitratos Dobutamina Milrinone

→ Nitroprusiato de sodio → Levosimendan o → → → →

0,25 – 5 µgrs/kg/min 1 – 10 mg/h 2 – 20 µgrs/kg/min Bolo: 25 – 75 µgrs/kg en 10 min Infusión: 0,25 – 1 µgrs/kg/min 0,3 – 5 µgrs/kg/min Bolo: 12 µgrs/kg en 10 min Infusión: 0,05 – 0,2 µgrs/kg/min

ANTICOAGULACIÓN: FA > 48 horas AP: Embolismo FA crónica AI > 55mm

3. CARDIOMIOPATÍA PERIPARTO 22 - 26 

MEDIDAS GENERALES

o Restricción hídrica (< 2Lts/día) o Restricción de sal (< 4grs/día) o Ejercicio físico moderado



MEDIDAS FARMACOLÓGICAS

o ORALES  → → → → →

PREPARTO Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos α Bloqueadores Bromocriptina?

POSTPARTO IECA – ARA Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos α Bloqueadores → Β Bloqueadores → Bromocriptina? 3 meses Postparto → Pentoxifilina? → Inmunosupresores: Biopsia (+) miocarditis 2 semanas sin respuesta al tratamiento convencional  → → → → →

o INTRAVENOSAS Síntomas severos – No respuesta a tratamiento VO → → → →

Dopamina Nitratos Dobutamina Milrinone

→ Nitroprusiato de sodio → Levosimendan

0,25 – 5 µgrs/kg/min 1 – 10 mg/h 2 – 20 µgrs/kg/min Bolo: 25 – 75 µgrs/kg en 10 min Infusión: 0,25 – 1 µgrs/kg/min 0,3 – 5 µgrs/kg/min Bolo: 12 µgrs/kg en 10 min Infusión: 0,05 – 0,2 µgrs/kg/min

o ANTICOAGULACIÓN → FE ≤ 35% → AP embolismo arterial – venoso → FA o ASITENCIA VENTRICULAR o BALÓN DE CONTRAPULSACIÓN INTRA AÓRTICO o TRASPLANTE CARDÍACO 4. DISFUNCIÓN VENTRICULAR 21,27 

MEDIDAS GENERALES

o Restricción hídrica (< 2Lts/día) o Restricción de sal (< 4grs/día)

o Ejercicio físico moderado 

MEDIDAS FARMACOLÓGICAS

o ORALES  → → → →

PREPARTO Hidralazina – Nitratos Digoxina Diuréticos α Bloqueadores

 → → → → →

POSTPARTO IECA – ARA Amlodipino – Hidralazina – Nitratos Digoxina Diuréticos α Bloqueadores Β Bloqueadores Bromocriptina?

→ →

o INTRAVENOSAS Síntomas severos – No respuesta a tratamiento VO → → → →

Dopamina Nitratos Dobutamina Milrinone

→ Nitroprusiato de sodio → Levosimendan

0,25 – 5 µgrs/kg/min 1 – 10 mg/h 2 – 20 µgrs/kg/min Bolo: 25 – 75 µgrs/kg en 10 min Infusión: 0,25 – 1 µgrs/kg/min 0,3 – 5 µgrs/kg/min Bolo: 12 µgrs/kg en 10 min Infusión: 0,05 – 0,2 µgrs/kg/min

● PRENATAL I.MANEJO TEMPRANO III NIVEL ¿Hospitalización el 3° trimestre: Cianosantes – HTP severa? II.TERMINACIÓN DEL EMBARAZO ¹ 14-20

    

Síndrome de Einsenmeger severo Cardiopatía congénita cianosante severa Hipertensión pulmonar severa Síndrome de Marfan Aorta > 4 cms Cardiomiopatía periparto con factores de mal pronóstico

III.ANTICOAGULACIÓN 1. CLASIFICACIÓN DEL RIESGO

TROMBOEMBÓLICO MATERNO

A. PRÓTESIS VALVULAR MECÁNICA 28-34 

RIESGO ALTO

o Prótesis valvulares de primera generación o Prótesis mecánica en posición Mitral o Prótesis mecánicas múltiples 

RIESGO “RELATIVAMENTE BAJO”

o Prótesis valvulares de segunda generación o Prótesis mecánica en posición Aórtica o FA – Flutter Auricular B. TROMBOEMBOLISMO VENOSO 35 -48 FACTORES DE RIESGO: o o o o o o o o

Edad > 35 años Obesidad IMC > 30 Trombofilia Parálisis MsIs Inmovilización Histerectomía obstétrica Preeclampsia ICC

C. APLAs 49 - 55 FACTORES DE RIESGO: o o o

Aborto precoz recurrente ( ≥ 3 ) Preeclampsia severa – recurrente RCIU 2. PRIORIDAD DE SEGURIDAD 59 - 64 MATERNA vs FETAL

➔

PROTESIS MECÁNICA



Riesgo Trombótico Alto SEGURIDAD MATERNA

o o o o

Warfarina 36 sems Terapia puente HBMM vs HNF Terapia puente HBMM vs HNF ASA 75 mgrs/d SEGURIDAD FETAL

o

HBMM – HNF 6 - 12 sems Warfarina hasta 36 sem Terapia puente HBMM vs HNF Terapia puente HBMM vs HNF ASA 75 MGRS/d



Riesgo Trombótico “Relativamente Bajo”

o o o o

SEGURIDAD MATERNA o o o o o

HBMM – HNF 6 - 12 sems Warfarina hasta 36 sem Terapia puente HBMM vs HNF Terapia puente HBMM vs HNF ASA 75 mgrs/d SEGURIDAD FETAL

o HBPM – HNF o ASA 75 mgrs/d ➔

TEV 65 - 67

1. Terapia puente HBPM vs HNF 2. AP TVP Tromboprofilaxis mecánica 3. Cesárea: FR 0 Movilización precoz FR 1 Tromboprofilaxis farmacológica vs mecánica FR ≥2 Tromboprofilaxis farmacológica y mecánica ➔

APLAs 68 -74

 

Aborto precoz recurrente – Perdida fetal tardía AP Neg. Tromboembolismos venosos – Arteriales Tromboprofilaxis farmacológica + ASA 75 mgrs/d

➔

PREECLAMPSIA 56 -58 75 -78

 

Alto riesgo: ASA 75 mgrs/d AP No Tromboprofilaxis farmacológica en los siguientes



3. TERAPIA PUENTE 79 - 85 A. HEPARINA NO FRACCIONADA (HNF) HNF 1. Monitoreo TPTa 2 veces 2. Monitoreo Anti Xa Profilaxis 0,1 – 0,25 U/ml Terapéuticas 0,35 – 0.7 U/ml

3. Dosis profilácticas: Suspender 2 – 4 h Pre Parto – Cx. 4. Dosis terapéuticas: Suspender 24 h Pre Parto – Cx. 5. Terapia puente 36 sems B. HEPARINA BAJO PESO MOLECULAR (HBPM) HBPM   

SIEMPRE Monitoreo Anti Xa Profilaxis 0,2 – 0,3 U/ml Terapéuticas 0,7 – 1,2 U/ml Dosis profilácticas: Suspender 12 h Pre Parto – Cx. Dosis terapéuticas: Suspender 24 h Pre Parto – Cx.

IV.DROGAS CARDÍACAS 86 -88          

    

1. SEGURAS Adenosina Amiloride Β Bloqueadores Bloqueadores del calcio Digoxina Flecainide Heparina Lidocaina Procainamida Quinidina 2. NO SEGURAS IECA Amiodarona Fenitoina Espironolactona Warfarina

V. LECTURA CRÍTICA – PLAN ECOCARDIOGRAFÍA  

Cambios fisiológicos Personal entrenado

PLAN 

➔ ➔ ➔ ➔ ➔ ➔

TODOS LOS PACIENTES Definir el tipo de lesión Estratificar clase funcional Clasificar el riesgo Definir consejería Consultas pertinentes Definir profilaxis de endocarditis bacteriana

 RIESGO INTERMEDIO – ALTO  Manejo multidisciplinario

     

Considerar intervenciones prenatales para optimización Socializar el plan de manejo Fomentar el parto vaginal Fomentar la Epidural temprana Fomentar el expulsivo asistido/fórceps terminal Evitar el cuidado postoperatorio subóptimo

VI.CIRUGÍA CARDIACA 1. CLASIFICACÍON DEL RIESGO 89 -92 A. RIESGO MATERNO FACTORES DE RIESGO     

Uso de drogas vasoactivas Edad > 35 años Cirugía emergente Reoperación Clase funcional NYHA IV

B. RIESGO FETAL 89 - 92 FACTORES DE RIESGO      

Edad > 35 años Cirugía emergente Reoperación Clase funcional NYHA IV Tipo de cardioplejia Tiempo de anoxia (Pinza Aórtica) Continuo Vs Intermitente 2. PROTECCIÓN FETAL - BCP 91 -95 13 – 28 sems

           

Monitorizar actividad uterina – FCF (>24s) Eco cardiografía fetal intraoperatoria Desviación uterina 15º lateral izquierdo Hematocrito materno > 28% Mantener saturación de oxigeno materna BCP Normotérmico BCP flujo alto (>2,5 L/min/m²) Presión de perfusión >70mm Hg Minimizar tiempo de BCP Flujo pulsátil Manejo ácido-base: α-stat Terapia tocolítica?? Perinatólogo y obstetra disponibles

• INTRAPARTO APROXIMACIÓN CON EL MÍNIMO

RIESGO

I. PROBLEMAS 96 -100 1. HEMORRAGIA → → → → →

→ →

A. ETIOLOGÍA Anticoagulación residual Policitemia Atonía uterina Obstétricas Quirúrgicas B. IMPLICACIONES Incapacidad para manejar la hipovolemia Edema pulmonar por sobrecarga hídrica 2. OXITOCICOS

A. OXITOCINA → ↓ PAM 30% - RVS 50% → ↑ GC 50% FC 20 – 30% VLat 20 – 30 % → Bolo: 5u + SS 0,9% 20cc en 5 – 10 min Infusión: 10u + SS 0,9% 500cc en 4 – 5 h B. ERGOMETRINE → HTA – HTP → Bolo: 250µgrs + SS 0,9% 20cc en 5 – 10 min C. MISOPROSTOL → No evaluada en enfermedad cardíaca → 200 – 800µgrs D. CARBOPROST → HTA – Colapso cardiovascular – edema pulmonar E. CARBETOCINA →

3. EDEMA PULMONAR

→ → → → → →

→ → → → →

A. ETIOLOGÍA Retención hídrica gestacional Redistribución hídrica compartimental Disfunción cardíaca Preeclapmsia Sobrecarga hídrica iatrogénica Farmacológica B. IMPLICACIONES Cuantificación estricta de líquidos Monitorizar volemia – diuresis Concentrar bolos e infusiones Vigilar la velocidad de infusión Utilizar equipos infusores 4. ARRITMIAS

A. ETIOLOGÍA → Menor umbral arritmogénico

→ → → → →

Tejido cardíaco cicatrizal Hipoxia – Hipercarbia Alteraciones metabólicas Trastornos hidro-electrolíticos Farmacológica

→ → → → → →

B. IMPLICACIONES Utilizar fármacos “relativamente” seguros Evaluar edad gestacional Disfunción ventricular aguda Desviación uterina lateral izquierda Riesgo de aspiración gástrica Cardioversión y desfibrilación convencionales 5. DISMINUCIÓN RVS

A. ETIOLOGÍA → Farmacológica → Técnica anestésica → Sepsis → → → → →

B. IMPLICACIONES Hipotensión arterial severa Aumento cortocircuito Síndrome de Einsenmeger Hipoflujo pulmonar: Hipoxemia Disfunción ventricular aguda 6. HIPERTENSIÓN PULMONAR AGUDA

A. ETIOLOGÍA → Defectos septales residuales → HTP primaria – secundaria → Hipoxia – Hipercarbia → Alteraciones metabólicas → Trastornos hidro-electrolíticos → Farmacológica B. IMPLICACIONES → Disfunción VD aguda → Isquemia VD → Eco cardiografía control 2° trimestre 7. TROMBOEMBOLISMO

→ → → → → →

A. ETIOLOGÍA Embarazo Prótesis valvular mecánica Anticuerpos antifosfolípidos Disfunción ventricular FA crónica AI > 55mm

→

Luxación uterina

B. IMPLICACIONES → Disfunción VD aguda → Embolismo sistémico: Cardíaco – Cerebral – → Sumergir el útero en SS 0,9%

Renal

8. ENDOCARDITIS BACTERIANA 5 A. FACTORES DE RIESGO → → → →

→ →

Prótesis valvulares mecánicas AP: Endocarditis bacteriana AP: Trasplante cardíaco y valvulopatía Enfermedad cardiaca congénita compleja Cianosante no corregida - derivaciones Corrección completa percutánea < 6 meses Corrección incompleta + comunicación residual B. INDICACIONES Sospecha de bacteremia: Odontológicos – Respiratorios (Toma de muestras) Parto vaginal – Cesárea OPCIONAL

C. FARMACOS → 30 -60 mins prequirúrgicos 1. Amoxacilina 2g VO 2. Ampicilina 2.0 g IM/IV o 3. Cefazolina/Ceftriazona 1gr IM/IV → Alérgicos 1. Cefalexina 2g VO o 2. Clindamicina 600mgrs VO/IV o 3. Azitromicina/Claritromicina 500mgrs VO o 4. Cefazolina/Ceftriazona 1gr IM/IV II. MANEJO DEL PARTO 1. MONITOREO

→ → → → → →

A. ASA vs Invasivo 101 - 106 Línea arterial Tipo de lesión Severidad de lesión Severidad de los síntomas Probabilidad de complicaciones Preeclampsia asociada Riesgo alto de sangrado

→ → → → →

Catéter venoso central Infusión drogas vasoactivas Riesgo alto de sangrado disfunción cardíaca Sensibilidad alto a hipovolemia: GC fijo Central vs Periférico

→ → → → → → → →

CATETER ARTERIA PULMONAR Evidencia Riesgos vs. Beneficios Estenosis Mitral o Aórtica severa Clase functional NYHA ≥ III Disfunción cardíaca Edema pulmonar refractario SDRA Preeclampsia + oliguria refractaria/edema pulmonar ECOCARDIOGRAFÍA TRANSESOFÁGICA → Anestesia general → Estenosis Mitral o Aórtica severa → Clase functional NYHA ≥ III → Disfunción cardíaca intraparto → Edema pulmonar refractario → SDRA → Preeclampsia + oliguria refractaria/edema pulmonar → Riesgo alto de sangrado → Sensibilidad alto a hipovolemia: GC fijo PICO – NICO → Estenosis Mitral o Aórtica severa → Clase functional NYHA ≥ III → Disfunción cardíaca intraparto → Edema pulmonary refractario → SDRA → Preeclampsia + oliguria refractaria/edema pulmonar

2. ANALGESIA 1,107 -109 ➔ Epidural vs. CSE ➔ Intravenosa: Remifentanil 3. VÍA DEL PARTO A. VAGINAL vs. Cesárea 1, 96, 98, 110-113 ➔ ➔ ➔ ➔

VAGINAL Epidural precoz Expulsivo asistido – fórceps terminal Posición semisentada Desviación 15° decúbito lateral izquierdo

→ → → → →

CESÁREA Indicación obstétrica Aortopatía > 4cms Aneurisma de Aorta Disección Aórtica Warfarina < 2sems 4. TÉCNICA ANESTÉSICA 1, 96, 98, 104, 107, 114-118

→ →         

A. General vs. Regional Estado cardiovascular Conceptos fisiopatológicos RIESGO DE COMPLICACIONES DISMINUCIÓN RVS – INOTROPISMO HIPERTENSIÓN PULMONAR (HTP) RIESGO TROMBOEMBÓLICO RIESGO DE MUERTE MATERNA VENTILACIÓN MECÁNICA POSTPARTO CIRUGÍA PROLONGADA PREDICTORES VÍA AÉREA DIFÍCIL PREFERENCIA DEL ANESTESIÒLOGO B. Obstétrica vs. Cardíaca PATOLOGÍAS ESTENÓTICAS

    

Resistencias vasculares sistémicas (RVS) Precarga – Postcarga Catecolaminas Frecuencia cardíaca Vía aérea C. REGIONAL

→ → → → → →

CSE – EPIDURAL “Titulada” Perdida de resistencia con SS 0,9% Monitoreo hemodinámico invasivo Desviación uterina 15° decúbito lateral izquierdo Evitar precarga - cocarga Umbral bajo para vasopresores: Fenilefrina ¿CONTRAINDICACIONES? “RELATIVAS” 119-121 → Coartación Aórtica severa no corregida → Tetralogía de Fallot no corregida → Síndrome de Einsenmeger → HTP primaria → Estenosis subaórtica hipertrófica idiopática

● POSTPARTO EVITAR CUIDADO SUB ÓPTIMO I. MONITOREO 1. SITIO A. UCI B. UCE C. UAD

2. TIEMPO Mínimo 72 horas 10 – 14 días A. SÍNDROME DE EINSENMEGER B. HTP severa II. ANALGESIA ÓPTIMA III. PLANIFICACIÓN 1. ANTICONCEPTIVOS ORALES COMBINADOS - CONTRAINDICADOS (RIESGO TROMBOSIS) 2. PROGESTERONA SOLA ORAL (CERAZETTE) – SEGURO Y EFECTIVO 3. PROGESTERONA SOLA IMPLANTE (IMPLANON) – SEGURO Y EFECTIVO 4. DISPOSITIVO INTRAUTERINO – PROFILAXIS Y REACCIÓN VASOVAGAL 5. TUBECTOMÍA – EMBOLISMO Y NEUMOPERITONEO IV. CONSEJERIA 1. Definir el nivel de ayuda en las tareas diarias 2. Capacitar para reconocimiento de los síntomas y signos de disfunción cardíaca 3. Elaborar un plan de nutrición: Dieta hiposódica – Hierro alto 4. En pacientes anticoaguladas: Capacitar para reconocimiento de los síntomas y signos de trombosis o sobre anticoagulación 5. Estimular la adherencia al tratamiento farmacológico

BIBLIOGRAFÍA ETAPA PRECONCEPCIONAL I. CLASIFICACIÓN DEL RIESGO 1. MATERNO ANATOMO-PATOLÓGICA 1. Lupton M, Oteng-Ntim E, Ayida G, Steer P J. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol 2002; 14: 137–143. ENFERMEDAD CONGÉNITA 2. CARPREG. Siu SC, Semer M et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation. 2001; 104:515-521. 3. Khairy DW, Ouyang, SM et al. Pregnancy outcomes in women with congenital heart disease. Circulation 2006; 113:517-524. 4. Drenthen W, Petronella G et al. Outcome of pregnancy in women with congenital heart disease. JACC 2007; 49-24:2303-2007. ENFERMEDAD VALVULAR 5. Robert O. Bonow, Blase A. Carabello et al. ACC/AHA Valvular heart disease Guidelines: 2008 Focused update incorporated. Circulation 2008; 118; e523-e661. CARDIOMIOPATIA PERIPARTO 6. Habli M, O´Brien T et al. Peripartum cardiomyopathy: Prognosis factors for long-term maternal outcome. Amer Journal of Obstet Gynecol October 2008 (Vol. 199, Issue 4, Pages 415.e1-415.e5). 7. Elkayan U, Tummala PP et al. Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy. N Engl J Med2001; 344: 1567-1571. 8. Lampert MB, Welnert L et al. Contractil reserve in patients with peripartum cardiomyopathy and recovered left ventricular function. Am J Obstet Gynecol 1997; 176: 189-195. 9. Ro A, Frishman W. Peripartum Cardiomyopathy. Cardiology in Review 2006; 14: 35–42

10. James PR. A review of peripartum cardiomyopathy. Int J Clin Pract.2004; 58: 363–365. 11. Elkayam U, Akhter MW, Singh H, et al. Pregnancy-associated cardiomyopathy. Clinical characteristics and a comparison between early and late presentation. Circulation. 2005; 111: 2050 –2055. 12. Ce´nac A, Djibo A, Chaigneau C, et al. Are anti-Chlamydia pneumoniae antibodies prognosis indicators for peripartum cardiomyopathy? J Cardiovasc Risk. 2003; 10: 195–199. 13. Sliwa K, Forster O, Zhanje F, et al. Outcome of subsequent pregnancy in patients with documented peripartum cardiomyopathy. Am J Cardiol. 2004;93: 1441–1443. 14. Elkayam U. Pregnant again after peripartum cardiomyopathy: to be or not to be? Eur Heart J. 2002; 23: 753–756. 15. Murali S, Baldisseri M, Peripartum cardiomyopathy. Crit Care Med 2005 Vol. 33, No. 10 (Suppl.) 16. Sliwa k, Forster O. Peripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectively studied patients. European Heart Journal (2006) 27, 441–446. 2. FETAL ENFERMEDAD CONGÉNITA 2. CARPREG. Siu SC, Semer M et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation. 2001; 104:515-521. 3. Khairy DW, Ouyang, SM et al. Pregnancy outcomes in women with congenital heart disease. Circulation 2006; 113:517-524. 4. Drenthen W, Petronella G et al. Outcome of pregnancy in women with congenital heart disease. JACC 2007; 49-24:2303-2007. 3. TRANSMISIÓN 1. Lupton M, Oteng-Ntim E, Ayida G, Steer P J. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol 2002; 14: 137–143. 9. Romano-Zelekha O, Hirsh R et al. The risk for congenital heart defects in offspring of individuals with congenital heart defects. Clin Genet 2001; 59: 325-329. 10. Burn J, Brennan P et al. Recurrence risk in offspring of adults with major heart defects; results from first cohort of British collaborative study. Lancet 1998; 351: 311-316. 11. Hyett J, Perdu M et al. Using fetal nuchal translucency to screen for major congenital cardiac defects at 10-14 weeks of gestation: population based cohort study. BMJ 1999; 318: 81-85. 12. Oberhansli I, Extermann P, Jaggi E, Pfizenmaier M. Fetal echocardiography in pregnancies of women with congenital heart disease: clinical utility and limitations. Thorac Cardiovasc Surg 2000; 48:323-327. 13. Carvalho JS. Early prenatal diagnosis of major congenital heart defects. Curr Opin Obstet Gynecol 2001; 13:155-159. 4. CONSEJERÍA 14. Lind J, Wallenburg HC. The Marfan syndrome and pregnancy: a retrospective study in a Dutch population. Eur JObstet Gynecol Reprod Biol 2001; 98:28-35. 15. Yentis SM, Steer PJ, Plaat F. Eisenmenger's syndrome in pregnancy: maternal and fetal mortality in the 1990s. Br J Obstet Gynaecol 1998; 105:921-92216. Elkayam U, Ostrzega E, Shotan A, Mehra A. Cardiovascular problems in Pregnant women with the Marfan syndrome. Ann Intern Med 1995; 123: 117-122. 17. Thorne SA. Pregnancy in heart disease. Heart 2004; 90: 450-456. 18. Gatzoulis Ma. Adult congenital heart disease: education, education, education. Nat Clin Pract Cardiovasc Med 2006; 3: 2-3. II. PLANIFICACIÓN 1. Lupton M, Oteng-Ntim E, Ayida G, Steer P J. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol 2002; 14: 137–143. 19. Leonard H, O'Sullivan JJ, Hunter S. Family planning requirements in the adult congenital heart disease clinic. Heart 1996; 76:60-62. 20. Arafeh J, Baird S. Curr Opin Obstet Gynecol 2002; 14: 137-43. Cardiac disease in pregnancy. Crit Care Nurs Q. 2006; 29:32–52. III. OPTIMIZACIÓN 1. LESIONES ESTENÓTICAS – 2- REGURGITANTES 5. Robert O. Bonow, Blase A. Carabello et al. ACC/AHA Valvular heart disease Guidelines: 2008 Focused update incorporated. Circulation 2008; 118; e523-e661. 21. Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003; 24: 761-781.

3. CARDIOMIOPATÍA PERIPARTO 22. Elkayam, U, Mohammed M et al. Pregnancy-Associated Cardiomyopathy Clinical Characteristics and a comparison Between Early and Late Presentation. Circulation. 2005; 111: 2050-2055. 23. Felker GM, Thompson RE, Hare JM, et al. Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med. 2000; 342: 1077–1084. 24. Denise G. Palmer. Peripartum Cardiomyopathy. J Perinat Neonat Nurs 2006; Vol. 20, No. 4, pp. 324– 332. 25. Dorbala S, Brozena S, Zeb S, et al. Risk stratification of women with peripartum cardiomyopathy at initial presentation: a dobutamine stress echocardiography study. J Am Soc Echocardiogr. 2005; 18: 45–48. 26. Chapa JB, Heiberger HB, Weinert L, DeCara J, Lang RM, Hibbard JU. Prognostic value of echocardiography in peripartum cardiomyopathy. Obstet Gynecol. 2005; 105: 1303–1308. 4. DISFUNCIÓN VENTRICULAR 21. Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003; 24: 761-781. 27. Dickstein, K. Cohen-Solal, A et al. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2008. European Heart Journal. 2008; 29: 2388-2442.

ETAPA PRENATAL II TERMINACIÓN DEL EMBARAZO 1. Lupton M, Oteng-Ntim E, Ayida G, Steer P J. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol 2002; 14: 137–143. 14. Lind J, Wallenburg HC. The Marfan syndrome and pregnancy: a retrospective study in a Dutch population. Eur JObstet Gynecol Reprod Biol 2001; 98:28-35. 15. Yentis SM, Steer PJ, Plaat F. Eisenmenger's syndrome in pregnancy: maternal and fetal mortality in the 1990s. Br J Obstet Gynaecol 1998; 105:921-92216. Elkayam U, Ostrzega E, Shotan A, Mehra A. Cardiovascular problems in Pregnant women with the Marfan syndrome. Ann Intern Med 1995; 123: 117-122. 17. Thorne SA. Pregnancy in heart disease. Heart 2004; 90: 450-456. 18. Gatzoulis Ma. Adult congenital heart disease: education, education, education. Nat Clin Pract Cardiovasc Med 2006; 3: 2-3. 19. Leonard H, O'Sullivan JJ, Hunter S. Family planning requirements in the adult congenital heart disease clinic. Heart 1996; 76:60-62. 20. Arafeh J, Baird S Curr Opin Obstet Gynecol 2002; 14: 137-43.. Cardiac disease in pregnancy. Crit Care Nurs Q. 2006; 29:32–52. III ANTICOAGULACIÓN 1. CLASIFICACIÓN DEL RIESGO PRÓTESIS VALVULAR MECÁNICA 28. Shannon M. Bates, Ian A. Creer et al. Venous Thromboembolism, Thrombophilia, Antithrombotic Therapy and Pregnancy. CHEST 2008; 133: 844S–886S. 29. Chan WS, Anand S, Ginsberg JS. Anticoagulation of pregnant women with mechanical heart valves: a systematic review of the literature. Arch Intern Med 2000; 160: 191–196. 30. Pavankumar P, Venugopal P, Kaul U, et al. Pregnancy in patients with prosthetic cardiac valves: a 10 years experience. Scand J Thorac Cardiovasc Surg 1988; 22: 19–22. 31. Al-Lawati AAM, Venkitraman M, Al-Delaime T, et al. Pregnancy and mechanical heart valves replacement: dilemma of anticoagulation. Eur J Cardiothorac Surg 2002; 22:223–227. 32. Schaefer C, Hannemann D, Meister R, et al. Vitamin K antagonists and pregnancy outcome: a multicentre prospective study. Thromb Haemost 2006; 95: 949–957. 33. Wesseling J, van Driel D, Heymans HAS, et al. Coumarins during pregnancy: long term effects on growth and development in school age children. Thromb Haemost 2001; 85: 609–613. 34. van Driel D, Wesseling J, Sauer PJJ, et al. In utero exposure to coumarins and cognition at 8 to 14 years old. Pediatrics. 2001; 107:123–129. TROMBOEMBOLISMO VENOSO 35. Lepercq J, Conard J, Borel-Derlon A, et al. Venous Thromboembolism during pregnancy: a retrospective study of enoxaparine safety in 624 pregnancies. Br J Obstet Gynaecol 2001; 108:1134–1140.

36. Lindqvist P, Dahlback B, Marsal K. Thrombotic risk during pregnancy: a population study. Obstet Gynecol 1999; 94: 595–599. 37. Andersen BS, Steffensen FH, Sorensen HT, et al. The cumulative incidence of venous thromboembolism during pregnancy and puerperium: an 11 year Danish population based study of 63,300 pregnancies. Acta Obstet Gynecol Scand 1998; 77: 170–173. 38. Simpson EL, Lawrenson RA, Nightingale AL, et al. Venous thromboembolism in pregnancy and the puerperium: incidence and additional risk factors from a London perinatal database. Br J Obstet Gynaecol 2001; 108: 56–60. 39. Kearon C. Risk factors for recurrent venous Thromboembolism and their implications for treatment. In: Lopez JA, Kearon C, Lee AYY, eds. Deep venous thrombosis: hematology; American Society of Hematology Education Program. Washington, DC: American Society of Hematology, 2004; 439–456. 40. Pabinger I, Grafenhofer H, Kyrle PA, et al. Temporary increase in the risk for recurrence during pregnancy in women with a history of venous thromboembolism. Blood 2002; 100:1060–1062. 41. Pabinger I, Grafenhofer H, Kaider A, et al. Risk of pregnancy associated venous thromboembolism in women with a history of venous thromboembolism. J Thromb Haemost 2005; 3: 949–954. 42. Greer IA. Thrombosis in pregnancy: maternal and fetal issues. Lancet 1999; 353:1258–1265 128 Robertson L, Wu O, Langhorne P, et al. for the Thrombosis Risk and Economic Assessment of Thrombophilia Screening (Treats) Study: thrombophilia in pregnancy; a systematic review. Br J Haematol 2005; 132: 171–196. 43. Biron-Andreani C, Schved J-F, Daures J-P. Factor V. Leiden mutation and pregnancy-related venous thromboembolism: What is the exact risk? Results from a meta-analysis. Thromb Haemost 2006; 95: 14–18. 44. Dizon-Townson D, Miller C, Sibai B, et al for the National Institute of Child Health and Human Development Maternal-Fetal Medicine Units Network. The relationship of the factor V Leiden mutation and pregnancy outcomes for mother and fetus. Obstet Gynecol 2005; 106: 517–524. 45. Middledorp S, Van der Meer J, Hamulyak K, et al. Counselling women with factor V Leiden homozygosity: use absolute instead of relative risks. Thromb Haemost 2001; 87: 360–361. 46. Martinelli I, Legnani C, Bucciarelli P, et al. Risk of pregnancy related venous thrombosis in carriers of severe inherited thrombophilia. Thromb Haemost 2001; 86: 800–803. 47. Friederich PW, Sanson B-J, Simioni P, et al. Frequency of pregnancy-related venous thromboembolism in anticoagulant factor-deficient women: implications for prophylaxis. Ann Intern Med 1996; 125: 955–960. 48. Gerhardt A, Scharf RE, Beckman MW et al. Prothrombin and factor V mutations in women with thrombosis during pregnancy and the puerperium. N Engl J Med 2000; 342: 374–380. APLAS 49. Hatasaka HH. Recurrent miscarriage epidemiologic factors, definitions, and incidence. Clin Obstet Gynecol 1994; 37: 625–634. 50. Brenner B. Clinical management of thrombophilia-related placental vascular complications. Blood 2004; 103: 4003–4009. 51. Greer IA. Thrombophilia: implications for pregnancy outcome. Thromb Res 2003; 109: 73–81. 52. Chamley LW, Dunclaf AM, Mitchell MD, et al. Action of anticardiolin and antibodies to 2-glycoprotein–I on trophoblast proliferation as a mechanism for fetal death. Lancet 1998; 352: 1037–1038. 53. Sthoeger ZM, Mozes E, Tartakovsky B. Anticardiolipin antibodies induce pregnancy failure by impairing embryonic implantation. Proc Natl Acad Sci U S A 1993; 90: 6565–6567. 54. Rand JH, Wu XX, Andree H, et al. Pregnancy loss in the antiphospholipid antibody syndrome: a possible thrombogenic mechanism. N Engl J Med 1997; 337: 154–160. 55. Kovalevsky G, Gracia CR, Berlin JA, et al. Evaluation of the association between hereditary thrombophilias and recurrent pregnancy loss: a meta-analysis. Arch Intern Med 2004; 164:558–563. PREECLAMPSIA 56. De Maat MP, Jansen MW, Hille ET, et al. Preeclampsia and its interaction with common variants in thrombophilia genes. J Thromb Haemost 2004; 2:1588–1593. 57. Mello G, Parretti E, Marozio L, et al. Thrombophilia is significantly associated with severe preeclampsia: results of a large-scale, case-controlled study. Hypertension 2005; 46: 1270–1274. 58. Morrison ER, Miedzybrodzka ZH, Campbell DM, et al. Prothrombotic genotypes are not associated with preeclampsia and gestational hypertension: results from a large population-based study and systematic review. Thromb Haemost 2002; 87: 779–785. 2. PRIORIDAD DE SEGURIDAD MATERNA vs. FETAL 59. Forestier F, Daffos F, Capella-Pavlovsky M. Low molecular weight heparin (PK 10169) does not cross the placenta during the second trimester of pregnancy: study by direct fetal blood sampling under ultrasound. Thromb Res 1984; 34: 557–560.

60. Forestier F, Daffos F, Rainaut M, et al. Low molecular weight heparin (CY 216) does not cross the placenta during the third trimester of pregnancy. Thromb Haemost 1987; 57:234 61. Lepercq J, Conard J, Borel-Derlon A, et al. Venous Thromboembolism during pregnancy: a retrospective study of enoxaparine safety in 624 pregnancies. Br J Obstet Gynaecol 2001; 108: 1134–1140. Hull RD, Delmore TJ, Carter CJ, et al. Adjusted subcutaneous heparin versus warfarin sodium in the longterm treatment of venous thromboembolism. N Engl J Med 1982; 306: 189–194. 62. Hull RD, Hirsh J, Jay R, et al. Different intensities of oral anticoagulant therapy in the treatment of proximal-vein thrombosis. N Engl J Med 1982; 307: 1676–1681. 63. Anderson DR, Ginsberg JS, Burrows R, et al. Subcutaneous heparin therapy during pregnancy: a need for concern at the time of delivery. Thromb Haemost 1991; 65: 248–250. 64. Monreal M, Lafoz E, Olive A, et al. Comparison of subcutaneous unfractionated heparin with low molecular weight heparin (Fragmin) in patients with venous Thromboembolism and contraindications to coumarin. Thromb Haemost 1994; 71: 7–11. TEV 65. Sanson BJ, Lensing AWA, Prins MH, et al. Safety of low-molecular-weight heparin in pregnancy: a systematic review. Thromb Haemost 1999; 81: 668–672. 66. Ellison J, Thomson AJ, Conkie JA, et al. Thromboprophylaxis following caesarean section: a comparison of the antithrombotic properties of three low molecular weight heparins; dalteparin, enoxaparine, and tinzaparin. Thromb Haemost 2001; 86: 1374 –1378. 66. Burrows RF, Gan ET, Gallus AS, et al. A randomized double-blind placebo controlled trial of low molecular weight heparin as prophylaxis in preventing venous thrombolic events after caesarean section: a pilot study. Br J Obstet Gynaecol 2001; 108: 835–839. 67. Gates S, Brocklehurst P, Ayers S, et al. on behalf of the Thromboprophylaxis in Pregnancy Advisory Group. Thromboprophylaxis and pregnancy: two randomized controlled pilot trials that used low-molecularweight heparin. Am J Obstet Gynecol 2004; 191: 1296–1303. APLAS 68. Farquharson RG, Quenby S, Greaves M. Antiphospholipid syndrome in pregnancy: a randomized, controlled trial of treatment. Obstet Gynecol 2002; 100: 408–413. 69. Triolo G, Ferrante A, Ciccia F, et al. Randomized study of subcutaneous low molecular weight heparin plus aspirin versus intravenous immunoglobulin in the treatment of recurrent fetal loss associated with antiphospholipid antibodies. Arthritis Rheum 2003; 48: 728–731. 70. Noble LS, Kutteh WH, Lashey N, et al. Antiphospholipid antibodies associated with recurrent pregnancy loss: prospective, multicenter, controlled pilot study comparing treatment with low-molecular-weight heparin versus unfractionated heparin. Fertil Steril 2005; 83: 684–690. 71. Stephenson MD, Ballem PJ, Tsang P, et al. Treatment of antiphospholipid antibody syndrome (APS) in pregnancy: a randomized pilot trial comparing low molecular weight heparin to unfractionated heparin. J Obstet Gynaecol Can 2004; 26: 729–734. 72. Brenner B, Hoffman R, Carp H, et al. Efficacy and safety of two doses of enoxaparine in women with thrombophilia and recurrent pregnancy loss: the LIVE-ENOX study. J Thromb Haemost 2005; 3: .227–229. 73. Walker ID, Kujovich JL, Greer IA, et al. The use of LMWH in pregnancies at risk: new evidence or perception? J Thromb Haemost 2005; 3: 778–793. 74. Lindqvist PG, Merlo J. Low molecular weight heparin for repeated pregnancy loss: is it based on solid evidence? J Thromb Haemost 2005; 3: 221–223. PREECLAMPSIA 75. Duley L, Henderson-Smart DJ, Knight M, et al. Antiplatelet agents for preventing pre-eclampsia and its complications: Cochrane Database Systematic Rev 2003; CD004659. 76. Hills FA, Abrahams VM, Gonzalez-Timon B, et al. Heparin prevents programmed cell death in human trophoblast. Mol Hum Reprod 2006; 12: 237–243. 77. Kalk JJ, Huisjes AJ, de Groot CJ, et al. Recurrence rate of pre-eclampsia in women with thrombophilia influenced by low-molecular-weight heparin treatment? Neth J Med 2004; 62: 83–87. 78. Mello G, Parretti E, Fatini C, et al. Low-molecular-weight heparin lowers the recurrence rate of preeclampsia and restores the physiological vascular changes in angiotensin converting enzyme DD women. Hypertens 2005; 45: 86–91. 3. TERAPIA PUENTE 79. Leyh RG, Fischer S, Ruhparwar A, et al. Anticoagulation for prosthetic heart valves during pregnancy: is low-molecular weight heparin an alternative. Eur J Cardiothorac Surg 2002; 21: 577–579.

80. Mahesh B, Evans S, Bryan AJ. Failure of low molecular weight heparin in the prevention of prosthetic mitral valve thrombosis during pregnancy: case report and review of options for anticoagulation. J Heart Valve Dis 2002; 11: 745–750. 81. Lev-Ran O, Kramer A, Gurevitch J, et al. Low-molecular weight heparin for prosthetic heart valves: treatment failure. Ann Thorac Surg 2000; 69:64–265. 82. Shapira Y, Sagie A, Battler A. Low-molecular-weight heparin for the treatment of patients with mechanical heart valves. Clin Cardiol 2002; 25: 323–327. 83. Oran B, Lee-Parritz A, Ansell J. Low molecular weight heparin for the prophylaxis of thromboembolism in women with prosthetic mechanical heart valves during pregnancy. Thromb Haemost 2004; 92: 747–751. 84. Turpie AGG, Gent M, Laupacis A, et al. A comparison of aspirin with placebo in patients treated with warfarin after heart-valve replacement. N Engl J Med 1993; 329: 524–529. 85. Crowther MA, Spitzer K, Julian J, et al. Pharmacokinetic profile of a low-molecular weight heparin (Reviparin) in pregnant patients: a prospective cohort study. Thromb Res 2000; 98: 133–138. IV DROGAS CARDÍACAS 1. SEGURAS 2. NO SEGURAS 86. Qasqas SA, McPherson C, Frishman WH, Elkayam U. Cardiovascular pharmacotherapeutic considerations during pregnancy and lactation. Cardiol Rev 004;12: 201-221. 87. Qasqas SA, McPherson C, Frishman WH, Elkayam U. Cardiovascular pharmacotherapeutic considerations during pregnancy and lactation. Cardiol Rev 2004;12: 240-261. 88. Steer PJ. Pregnancy and contraception. In: Gatzoulis MA, Swan L, Therrien J, Pantely GA, eds. Adult congenital heart disease: A practical guide. Oxford: BMJ Publishing, Blackwell Publishing, 2005:16-35. V. PLAN – LECTURA CRÍTICA VI. CIRUGÍA CARDÍACA 1. CLASIFICACIÓN DEL RIESGO MATERNO – FETAL 89. Arnoni R, Arnoni A et al. Risk Factors Associated With Cardiac Surgery During Pregnancy. Ann Thorac Surg 2003; 76: 1605– 1608. 90. Weiss BM, von Segesser LK, Alon E, Seifert B, Turina MI. Outcome of cardiovascular surgery and pregnancy: a systematic review of the period 1984–1996. Am J Obstet Gynecol 1998; 179: 1643–1653. 91. Chandrasekhar S Cook C, DO et al. Cardiac Surgery in the Parturient. Anesth Analg 2009; 108: 777– 785. 92. Patel A, Asopa S et al. Cardiac Surgery during Pregnancy. Tex Heart Inst J 2008; 35(3): 307-12. 2. PROTECCIÓN FETAL - BCP 91. Chandrasekhar S Cook C, DO et al. Cardiac Surgery in the Parturient. Anesth Analg 2009; 108: 777– 785. 92. Patel A, Asopa S et al. Cardiac Surgery during Pregnancy. Tex Heart Inst J 2008; 35(3): 307-12. 93. Karahan N, Ozturk T, Yetkin U, Yilik L, Baloglu A, Gurbuz A. Managing severe heart failure in a pregnant patient undergoing cardiopulmonary bypass: case report and review of the literature. J Cardiothorac Vasc Anesth 2004;18: 339–43 94. Iscan ZH, Mavioglu L, Vural KM, Kucuker S, Birincioglu L. Cardiac surgery during pregnancy. J Heart Valve Dis 2006; 15:686–690. 95. Pomini F, Mercogliano D, Cavalletti C, Caruso A, Pomini P. Cardiopulmonary bypass in pregnancy. Ann Thorac Surg 1996; 61:259–268.

ETAPA INTRAPARTO I. PROBLEMAS 1. – 7. 96. Dob D, Yentis S. Practical management of the parturient with congenital heart disease. International Journal of Obstetric Anesthesia 2006; 15, 137–144. 97. Oakley C. Management of labour and delivery in the high risk patient. In: Oakley C, ed. Heart disease in pregnancy. London: BMJ Publishing Group, 1997: 375–379. 98. Uebing a, Steer PJ, Yentis S and Gatzoulis M. Pregnancy and congenital heart disease. BMJ 2006; 332; 401-406. 99. Siu S, Colman J. Heart disease and pregnancy. Heart 2001; 85: 710-715. 100. Chow T, Galvin J, McGovern B. Antiarrhythmic drug therapy in pregnancy and lactation. Am J Cardiol 1998; 82: 58I–62I.

8. ENDOCARDITIS BACTERIANA 5. Robert O. Bonow, Blase A. Carabello et al. ACC/AHA Valvular heart disease Guidelines: 2008 Focused update incorporated. Circulation 2008; 118; e523-e661. II. MONITOREO 1. ASA vs. INVASIVO 101. Dob D P, Yentis S M. UK Registry of high-risk obstetric anaesthesia: report on cardiorespiratory disease. Int J Obstet Anesth 2001; 10: 267–272. 102. Lewis N, Dob D P, Yentis S M. UK Registry of high-risk obstetric anaesthesia: arrhythmias, cardiomyopathy, aortic stenosis, transposition of the great arteries and Marfans syndrome. Int J Obstet Anesth 2003; 12: 28–34. 103. Oakley C. Management of labour and delivery in the high risk patient. In: Oakley C, ed. Heart disease in pregnancy. London: BMJ Publishing Group, 1997: 375–379. 104. O´Hare R, McLoughlin C, Milligan K, McNamee D, Sidhu H. Anaesthesia for caesarean section in the presence of severe primary pulmonary hypertension. Br J Anaesth 1998; 81: 790–792. 105. Palmer C M, DiNardo J A, Hays R L, Van Maren G A. Use of transesophageal echocardiography for delivery of a parturient with severe pulmonary hypertension. Int J Obstet Anesth 2002; 11: 48–51. 106. Penning S, Robinson KD, Major CA, Garite TJ. A comparison of echocardiography and pulmonary artery catheterization for evaluation of pulmonary artery pressures in pregnant patients with suspected pulmonary hypertension. Am J Obstet Gynecol 2001; 184: 1568-1570. 2. ANALGESIA 107. Suntharalingam G, Dob D, Yentis S M. Obstetric epidural analgesia in aortic stenosis: a low-dose technique for labour and instrumental delivery. Int J Obstet Anesth 2001; 10: 29–134. 108. Van de Velde M, Budts W, Vandermeersch E, Spitz B. Continuous spinal analgesia for labor pain in a parturient with aortic stenosis. Int J Obstet Anesth 2003; 12: 51–54. 109. Kee W D, Shen J, Chiu A T, Lok I, Khaw K S. Combined spinal epidural analgesia in the management of labouring parturients with mitral stenosis. Anaesth Intensive Care 1999; 27: 523–526. 3. VÍA DEL PARTO VAGINAL vs Cesárea 1. Lupton M, Oteng-Ntim E, Ayida G, Steer P J. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol 2002; 14: 137–143. 96. Dob D, Yentis S. Practical management of the parturient with congenital heart disease. International Journal of Obstetric Anesthesia 2006; 15, 137–144. 98. Uebing A, Steer P, Yentis S, Michael A Gatzoulis. Pregnancy and congenital heart disease. BMJ 2006;332: 401–406. 110. Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med 1981; 71: 784-790. 111. Elkayam U, Ostrzega E, Shotan A, Mehra A. Cardiovascular problems in pregnant women with the Marfan syndrome. Ann Intern Med 1995; 123: 117-122. 112. Rossiter JP, Repke JT, Morales AJ, et al. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol 1995; 173: 1599-1606. 113. Yentis S, Gatzoulis M A, Steer P. Pregnancy and coarctation of the aorta. J R Soc Med 2003; 96: 471. 4. TÉCNICA ANESTÉSICA 1. Lupton M, Oteng-Ntim E, Ayida G, Steer P J. Cardiac disease in pregnancy. Curr Opin Obstet Gynecol 2002; 14: 137–143. 96. Dob D, Yentis S. Practical management of the parturient with congenital heart disease. International Journal of Obstetric Anesthesia 2006; 15, 137–144. 98. Uebing A, Steer P, Yentis S, Michael A Gatzoulis. Pregnancy and congenital heart disease. BMJ 2006; 332: 401–406. 104. O´Hare R, McLoughlin C, Milligan K, McNamee D, Sidhu H. Anaesthesia for caesarean section in the presence of severe primary pulmonary hypertension. Br J Anaesth 1998; 81: 790–792. 107. Suntharalingam G, Dob D, Yentis S M. Obstetric epidural analgesia in aortic stenosis: a low-dose technique for labour and instrumental delivery. Int J Obstet Anesth 2001; 10: 129–134. 114. Velickovic IA, Leicht CH. Continuous spinal anesthesia for cesarean section in a parturient with severe recurrent peripartum cardiomyopathy. Int J Obstet Anesth. 2004;13: 40–43. 115. Connelly NR, Chin MT, Parker RK, et al. Pregnancy and delivery in a patient with recent peripartum

cardiomyopathy. Int J Obstet Anesth. 1998;7: 38–41. 116. Pirlet M, Baird M, Pryn S, et al. Low dose combined spinal– epidural anaesthesia for caesarean section in a patient with peripartum cardiomyopathy. Int J Obstet Anesth. 2000;9: 189 –192. 117. Shnaider R, Ezri T, Szmuk P, et al. Combined spinal– epidural anesthesia for cesarean section in a patient with peripartum dilated cardiomyopathy. Can J Anaesth. 2001;48: 681– 683. 118. McCarroll CP, Paxton LD, Elliott P, et al. Use of remifentanil in a patient with peripartum cardiomyopathy requiring caesarean section. Br J Anaesth. 2001; 86: 135–138. “CONTRAINDICACIONES RELATIVAS” 119. Gomar C, Errando C. Neuroaxial anaesthesia in obstetrical patients with cardiac disease. Current Opinion in Anaesthesiology 2005, 18: 507–512. 120. Kuczkowski KM. Labor analgesia for the parturient with an uncommon disorder: a common dilemma in the delivery suite. Obstet Gynecol Surg 2003; 58: 800–803. 121. Martin JT, Tautz TJ, Antognini JF. Safety of regional anesthesia in Eisenmenger’s syndrome. Reg Anesth Pain Med 2002; 27:509–513. 22 Walker E, Malins AF. Anaesthetic management of aortic coarctation in pregnancy. Int J Obstet Anesth 2002; 11: 156–159.

PULMONARY ARTERIAL HYPERTENSION IN CONGENITAL HEART DISEASE AND PREGNANCY: ANESTHESIC MANAGEMENT

DIAGNOSTIC AND ASSESMENT SCREENING When pulmonary hypertension (PH) is suspected based on medical history, risk factor assessment, physical examination, chest x-ray (CXR) and electrocardiogram, an echocardiogram is indicated. PH refers to the presence of a mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg at rest, in the setting of a normal pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg. The diagnosis of pulmonary arterial hypertension (PAH), remains a diagnosis of exclusion. After excluding lung disease, thromboembolic disease, LV disease or valvular disease the diagnosis criteria for PAH requires confirmation with a right heart catheterization (RHC): mPAP > 25 mm Hg, a normal PCWP and a pulmonary vascular resistance > 3 Wood units is also required. The transpulmonary gradient (mPAPPCWP) is significantly elevated in PAH. (1, 2) Idiopathic PH (IPH) requires acute vasodilator testing, which involves the administration of pharmacologic agents to test the presence of pulmonary vasoreactivity, which identifies patients with a better prognosis and potential candidates for long term calcium channel blockers therapy. The definition of acute responders is a reduction in mPAP of ≥10 mm Hg to an absolute mPAP < 40 mm Hg, without a decrease in cardiac output (CO). In the patient with an mPAP < 40 mm Hg at baseline, a substantial (> 20%) reduction in mPAP it would be reasonable to administer a trial of calcium channel blockers and assess the clinical response. Those with overt RH failure, significantly elevated left heart filling pressures or hemodynamic instability should not undergo this test. (3, 4)

GENERAL RECOMMENDATIONS (5) 1. Regular follow up in referral centers with assessment of functional class and exercise capacity 2. Avoid unnecessary non cardiac surgery or perform it in referral center with trained anesthesia and cardiology staff 3. A multidisciplinary approach is recommended 4. Recommendations for physical activity: Avoid strenuous exercise 5. Genetic counseling: current guidelines recommend that pregnancy be avoided or terminated early in women with PAH (6) 6. Maintain fluid balance and avoid either dehydration or fluid overload 7. Annual immunization for influenza - pneumococcal 8. Diuretics: In patient with right ventricular (RV) failure with fluid overload 9. Digitals: In patient with RV failure 10. Oxygen therapy: Only in patients in whom it produces a consistent increase in arterial oxygen saturation > 90% 11. Anticoagulation: INR of 1.5 to 2.5. Increased pulmonary embolism (PE) risk – Hyperviscosity syndrome - IPH 12. Hyperviscosity syndrome must be ruled out. (7). Box 1. 13. Classify according to World Health Organization (WHO) functional status scale. (8) • I: No limitation of usual physical activity • II: Normal physical activity causes increased dyspnea, fatigue, chest pain or presyncope. • III: Less than ordinary activity causes increased dyspnea, fatigue, chest pain or presyncope • IV: Unable to perform any physical activity at rest; signs of RV failure; dyspnea and/or fatigue may be present at rest; symptoms increased by almost any physical activity 14. Prognostic factors (9) • Clinical evidence of RV failure

• • • • • • • • •

Rapid progression symptoms WHO class IV 6 minute walk (6MK) distance < 300 m Cardiopulmonary exercise testing (CPET) Peak VO2 < 10.4 ml/kg/min – Peak systemic blood pressure < 120 mm Hg Echocardiography: Pericardial effusion, significant RV enlargement/dysfunction, right atrial enlargement Magnetic Resonance: RV dysfunction: Stroke volume ≤ 25mL/m² - RV end diastolic volume ≥ 84mL/m² - LV end diastolic volume ≤ 40mL/m² Hemodynamics: RAP > 20 mm Hg, CI < 2.0 L/min/m² Brain natriuretic peptide (BNP) Significantly elevated Scleroderma spectrum of diseases

Point of Care: (POC). Peripartum special considerations: 1. Prophylaxis to prevent bacterial endocarditis in patients at high risk for adverse outcomes: (10, 11) Class IIa Patients with prosthetic cardiac valve or prosthetic material used for cardiac valve repair Level of evidence B Patient with previous infective endocarditis. Level of evidence B Patient with Congenital heart disease (CHD). • Unrepaired cyanotic CHD, including palliative shunts and conduits. Level of evidence B • Completely repaired congenital heart defects repaired with prosthetic material or device. Level of evidence B • Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (both of which inhibit endothelialization. Level of evidence B Cardiac transplant recipients with valve regurgitation due a structurally abnormal valve. Level of evidence C Class III It is not recommended for nondental procedures (such as TEE, endoscopy or colonoscopy) in the absence of active infection. Level of evidence B 2. Anticoagulation: Prophylactic vs. full anticoagulation doses 3. Avoid arrhythmias 4. Avoid hypoxia 5. Maximize systemic vascular resistance (SVR) 6. Minimize increase pulmonary vascular resistance (PVR) 7. Invasive monitoring: Arterial line – CVP – PAC?? – Cardiac output 8. General Vs Regional anesthesia 9. Uterotonics for uterine atony prevention 10. Postpartum care: monitor for PH rebound and increased risk for thromboembolism

PERIOPERATIVE MANAGEMENT GOALS: (12, 13) 1. Maximize SVR and blood pressure (BP) 2. Minimize increases PVR 3. Optimize oxygenation: SatO2 > 90% - PaO2 > 70 mm Hg 4. Maintenance of cardiac contractility 5. Maintenance sinus rhythm 6. Optimize preload volume without overload 7. Avoid Aorto-Caval compression syndrome (left lateral decubitus) is the position of choice.7.

GENERAL APPROACH FOR ALL PATIENTS 1. Aspiration pulmonary prophylaxis: Metoclopramide 10 mg IV. Ranitidine 50 mg IV 2. Mechanical Thromboembolic prophylaxis: Graduated compression stockings – Pneumatic intermittent compression 3. Endocarditis prophylaxis: Ampicilin 2 grs IV, Gentamicin 80 mg IV, Ampicilin 1 gr IV 6hours after. 4. Monitoring: • Basic: NIBP- EKG (5 leads) – Oxygen saturation - Urinary output – DC Thoracic adhesive pads – Neuromuscular function – Temperature - BIS index (general anesthesia)– ETCO2 (general anesthesia)– Fetal cardiac monitoring – Fluids balance • Invasive: Central venous catheter: Either 8.5 F Swan-Ganz sheath - Arterial line # 20 • Cardiac output: i.e. PIcCO – LIDCO – TEE (general anesthesia) 5. Active warming: Fluids warmer infuser – Convective warming blankets – Water convector mattress 6. Conservative fluid management. 7. Avoid air injection in patients with shunts 8. Enoxaparine neutralization: The following approach is recommended in clinical situations where the anticoagulant effect of LMWH needs to be neutralized. If LMWH was given within 8 h, protamine sulfate should be administered in a dose of 1mg per 100 anti-Xa units of LMWH (1 mg enoxaparine equals approximately 100 anti-Xa units). A second dose of 0.5 mg protamine sulfate per 100 anti-Xa units should be administered if bleeding continues. Smaller doses of protamine sulfate can be given if the time since LMWH administration is longer than 8 h (14). PH it´s a major concern with protamine use, be careful, risk benefit as always it´s necessary. 9. Vasopressor drugs: Phenylefrine either boluses or infusion. Noradrenaline or vasopressin are others options. For either RV failure or acute PH rebound (CVP increase, hypoxemia); although there are no outcome data to support any therapeutic strategy for RV failure when PVR is elevated; the combination of inhaled iloprost or intravenous milrinone with oral sildenafil produces a synergistic reduction in PVR, while sparing systemic vascular resistance. Levosimendan is a promising new inotrope for the treatment of RV failure, although its role in comparison to older agents such as dobutamine, adrenaline and milrinone has yet to be determined. This is also the case for the use of vasopressin as an alternative pressor to noradrenaline. If all else has failed, mechanical support of the RV should be considered in selected cases. (15) 10. Therapeutic strategies for PH patient are: a. Oxygen probably is the easiest and simplest way. b. Sildenafil is a good option. (16-18)

c. Bosentan is usually stopped whilst pregnant, as it maybe teratogenic. The FDA requires that lever function tests be checked monthly and the hematocrit should be checked every 3 months. Hormonal methods of birth control may be less effective with concurrent administration of bosentan and barrier techniques of contra caption are recommended. d. Intravenous epoprostenol is the only therapy for PAH that has been shown to prolong survival. It improves functional class, exercise tolerance and hemodynamics. An open label, randomized trial of 81 functional class III and IV IPAH patients demonstrated significant improvement in the primary end point of 6MW test (32 m increase with epoprostenol versus 15 m decrease with conventional therapy alone, placebo-corrected change of 47 m) and in secondary end points including hemodynamics and quality of life. (19) e. Prostaciclin can be used IV (anticoagulation issue) or as nebulizer to control symptoms, rises in PA pressure and in a perioperative arrest situation it will use as first line treatment. (20) In the acute situation, inhaled nitric oxide may be of benefit. Its ease of administration, tolerability and beneficial effects of reducing PVR and improving right ventricular function, thereby enhancing the ability of the right ventricle to compensate for the physiological changes of pregnancy, are appealing. Furthermore there may be an indirect benefit on left ventricular performance. As with other pulmonary vasoactive compounds, there are limited data and no prospective randomized trials of inhaled nitric oxide in pregnancy. (21,22) 11. Uterine atony prophylaxis: a. Oxytocin: Effective uterine contraction can be achieved after elective caesarean section in non-laboring women at term by administering boluses no larger than 1IU, the minimum effective intravenous bolus dose of oxytocin being 0.35 IU, while the necessary dose in laboring women at caesarean delivery is about nine times higher (23). The difference is believed to be due to the reduction of oxytocin-binding sites and desensitization of myometrial oxytocin receptors in active labor. Hemodynamic alterations will depend on the amount of vasopressin available (24). It will be harmful for cardiac patient; generally primary pulmonary hypertensives are more sensitive than secondary cardiac case. Oxytocin may occasionally induce hypotension, tachycardia and increase in cardiac output (CO). Hypotension is most likely due to vasodilatation as receptors are also present in vascular smooth muscles and increases in heart rate and CO are believed to be sings of compensatory mechanism. If use, some groups give a small dose (e.g. 1 IU) as infusion over 20 minutes. (25) b. Ergometrine is contraindicated by side effects like hypertension (pulmonary and systemic), coronary artery spasm and bronchospasm. c. Syntometrine, a mixture of % UI oxytocin and 0.5 mg ergometrine, is associated with a reduction of postpartum hemorrhage, compared with oxytocin alone and however is associated with more adverse effects. Carbetocine is a good choice, single dose, decreased incidence of side effects (nausea, vomiting and hypertension) and long lasting oxytocin agonist analog. The most commonly used prostaglandin is PGE1. It has limited side effects with respect to the cardiovascular system, both after vaginal delivery and after caesarean section. The effect is comparable to that of IV oxytocin for the prevention of postpartum hemorrhage PPH. The administration of PGF2-α has been reported to result in pulmonary edema during a caesarean section and maternal arterial desaturation with an increase in intrapulmonary shunting in women with severe uterine atony. An overdose of PGF2-α has

been reported to produce cardiovascular collapse and severe bronchospasm and hypotension.

SPECIFIC APPROACH VAGINAL DELIVERY Labor and delivery must be planned carefully and well in advance. Ideally decisions about timing and mode should be agreed after a multidisciplinary case discussion including the patient. These decisions must be communicated appropriately to the patient and the healthcare team, anesthesiologists, obstetricians, pediatricians and cardiologists; it includes patient chart, all tests and the recommended delivery plan. Vaginal delivery carries a lower risk of complications for both the mother and the fetus. It causes smaller shifts in blood volume, less hemorrhage, fewer clotting complications and fewer infections. However, prolonged and difficult labor should be avoided and detailed continuous monitoring of the mother and fetus is mandatory but cesarean section rates are needed in 30 – 36% of the cases. (26-28) Low dose epidural analgesia is recommended for virtually all women with cardiac disease in labor, an exception being those receiving therapeutic anticoagulation. The advantages of regional analgesia usually outweigh the relatively small risk of epidural hematoma when prophylactic heparin is used; by providing adequate analgesia and avoiding further increases in cardiac output from pain and anxiety. The need for more invasive monitoring (e.g. arterial lines) is determined by the severity of the cardiac condition. PACs could be harmful: (arrhythmias, fatal pulmonary artery rupture from wedging, and questionable information). If there is a need of cardiac output monitoring a non-invasive device like PIcCO or LID CO/pulse contour analysis should be preferred. The second stage should be shortened with elective assisted delivery, with terminal forceps, to avoid maternal effort. Solutions containing bupivacaine < 0.1% with fentanyl 2-5 µg/mL provide good hemodynamic stability, even in those with fixed output states. We have found no advantage of epidural infusions over boluses of 5-15 mL. Intrathecal opioids without local anesthetics have been described, either as a single injection or as the initial part of a combined epidural-spinal or continuous spinal technique, although we have had no experience as we have found the epidural regimen adequate. Since women with severe disease are usually admitted to hospital in the third trimester, there is enough time to insert the epidural catheter either in early labor or before labor is induced, so that they are in minimal discomfort. 1. Analgesia: Early low dose epidural analgesia • Neuroaxial: Test dose: Don´t use adrenaline by adrenergic effect (tachycardia and hypertension). Epidural catheter: Place it “Early” under saline loss of resistance technique. (If it´s a patient intracardiac shunts). 1) Combined spinal epidural (CSE): Spinal analgesia: _ Bupivacaine 0,5% _ Fentanyl Afterwards use an epidural catheter, titrating for a T10 level with bupivacaine < 0.1% without adrenaline. 2) Epidural: For a T10 level _Bupivacaine < 0.1% _ Fentanyl 25 mcg 3) Spinal catheter:

• IV: PCA + Infusion Remifentanyl 0.05 – 0.2 mcg/kg/min Dexmedetomidine 0.2 – 1 mcg/kg/hour. It could be controversial by fetus effects GENERAL ANESTHESIA EMERGENT CESAREAN SECTIO AND ANTICOAGULATED PATIENT IV INDUCTION 1. Preoxygenation: High flow with Oxygen 100% 2. Midazolam 1-2 mg 3. Etomidate 0.3 mg/kg 4. Remifentanyl 80-160 mcg or Fentanyl 500- 100 mcg 5. Rocuronio 0.6 mgr/kg or Succinilcoline 1.5 mgr/kg 6. Orotracheal intubation: Size 6.5 – 7 MAINTENANCE AFTER UMBILICAL CORD CLAMP (DOUBLE CLAMPING – ARTERIAL BLOOD GASES) 1. 2. 3. 4. 5. 6.

Oxygen 100 %. Don’t use N2O. Remifentayl 0.05-0.2 mcg/kg/min. End tidal gases expiration < 1 MAC (Risk of Uterine atony) Dexmedetomidine 0.2-1 mcg/kg/hour Morphine 3-5 mg Get most experienced obstetrician to do section. Perhaps consider a prophylactic BLynch suture. 7. Analgesia: IV PCA Morphine, NSAID´s IV, PO Acetaminophen 8. Neuromuscular relaxants antagonism assessed by peripheral nervous stimulator. 9. Awake extubation: Adrenergic response attenuated with either remifentanyl or dexmedetomidine 10. Beware of 10 days postoperative monitoring. We recommended 72 hours at ICU and then 4-7 days in other critical care facility with a central line. REGIONAL ANESTHESIA 1. Complete anticoagulation screening: PT, aPTT, Platelets and anti-Xa units before surgery. Verify a normal range, for medical legal issue about epidural hematoma risk. 2. Anesthesia technique: • Combined spinal epidural (CSE) Place the epidural catheter under lost of resistance technique with saline. (If it´s a shunt patient). Don´t use adrenaline for dose test by adrenergic effect (tachycardia and hypertension). Never use a single shot spinal anesthesia Spinal anesthesia: _ Bupivacaine _ Fentanyl _ Morphine And then by epidural catheter look for a T4-T6 level with Lidocaine 2% without adrenaline. • Spinal catheter: _ Bupivacaine 0,5% _ Fentanyl _ Morphine

•

3. 4. 5.

And then by spinal catheter look for a T8 level with bupivacaine 0,5% Epidural anesthesia: Place the epidural catheter under lost of resistance technique with saline. (If it´s a shunt patient). _ Levo- Bupivacaine/Ropivacaine 0.75 % or Lidocaine 2% without adrenaline for a T4-T6 level _ Fentanyl 100 mcg _ Morphine 2 mg Get most experienced obstetrician to do section. Perhaps consider a prophylactic BLynch suture. Analgesia: Epidural bupivacaine /ropivacaine PCA, Epidural/spinal morphine, NSAID´s IV, PO Acetaminophen. Beware of 10 days postoperative monitoring. We recommended 72 hours at ICU and then 4-7 days in other critical care facility with a central line.

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