ACC-2017 Pregnancy - CHD

Template:Pregnancy -CHD - 2017 Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Associate Editor(s)-in-Chief: Arzu Kalayci, M.D. [2]

Management of Pregnancy in Patients With Complex Congenital heart Disease

Modified Who Classification of Maternal Cardiovascular Risk

Who Pregnancy Risk Category	Risk Description	Maternal Risk Factors
I	No detectable increase in maternal mortality and no/mild increase in morbidity risk	Uncomplicated small/mild pulmonary stenosis, PDA, mitral valve prolapse; Successfully repaired simple lesions (ASD, VSD, PDA, anomalous pulmonary venous drainage); Atrial or ventricular ectopic beats, isolated
II	Small increase in maternal mortality and moderate increase in morbidity risk	If otherwise well and uncomplicated: Unoperated ASD, VSD; Repaired TOF; Most arrhythmias
II–III	Moderate increase in maternal mortality morbidity risk	Mild LV impairment; Hypertrophic cardiomyopathy; Native or tissue valvular disease (not considered risk category I or IV); Marfan syndrome without aortic dilation; Aortic dilation <45 mm in bicuspid aortic valve aortopathy; Repaired coarctation
IV	Extremely high maternal mortality or severe morbidity risk. Pregnancy is contraindicated. In the event of pregnancy, termination should be discussed. If pregnancy continues, care should follow class III recommendations.	Pulmonary arterial hypertension (of any cause); Severe systemic ventricular dysfunction (LV ejection fraction <30%, NYHA class III-IV); Previous peripartum cardiomyopathy with any residual impairment of LV function; Severe mitral stenosis, severe symptomatic aortic stenosis; Aortic dilation >45 mm in Marfan syndrome; Aortic dilation >50 mm in bicuspid aortic valve aortopathy; Native severe coarctation
AS indicates aortic stenosis; ASD, atrial septal defect; CHD, congenital heart disease; LV, left ventricular; NYHA, New York Heart Association; PDA, patent ductus arteriosus; RV, right ventricle; TOF, tetralogy of Fallot; VSD, ventricular septal defect; and WHO, World Health Organisation.

Medications During Pregnancy

	Common Examples	FDA Pregnancy Category	Teratogenic risks (First Trimester)	Other Pregnancy Concerns (Second and Third Trimesters)	Suggested Evaluation	Lactation	Notes
Antihypertensives
β-Blockers	Metoprolol Propranolol Carvedilol Atenolol	C C C D	None reported	Possible association with fetal growth restriction in second and third trimesters (atenolol, propranolol), neonatal bradycardia (esmolol, nadolol)	Consider serial fetal sonography to assess interval fetal growth in second and third trimesters	Except for atenolol, probably safe
Combined α-/β- blockers	Labetalol	C	None reported	No		Yes
Calcium channel blockers	Nifedipine Verapamil Diltiazem	C	None reported	Caution if used in combination with magnesium sulfate		Yes
ACE inhibitors	Captopril Enalapril Lisinopril	C ( first trimester) D (second and third trimesters)		Fetal renal dysplasia, oligohydramnios, growth restriction, and intrauterine demise reported in second and third trimesters	Consider fetal echocardiography with rst trimester exposure, serial sonography to assess interval fetal growth and amniotic fluid volume in second and third trimesters	Yes (captopril and enalapril) Unknown (lisinopril)	Avoid if possible during pregnancy
α2- Adrenergic agonists	Methyldopa Clonidine	B C	None reported	Induction of positive indirect Coombs testing (methyldopa), rare neonatal hypertension (clonidine)		Yes (methyldopa) Probably safe (clonidine)
Vasodilators	Hydralazine Epoprostenol Nitroprusside Nitroglycerin Isosorbide dinitrate Sildenafil	C B B	None reported	Possible transient fetal bradycardia (nitroprusside)		Yes (hydralazine) Probably safe (nitroglycerin) Unknown (epoprostenol, nitroprusside, isosorbide dinitrate, sildenafil)
Diuretics
Thiazide diuretics	Hydrochlorothiazide	B	None reported	Possible increased risk of neonatal hypoglycemia and thrombocytopenia	Consider neonatal blood count and electrolyte evaluation	Yes (AAP) No (WHO)	Maintenance therapy permissible, but therapy not usually started during pregnancy
Loop of Henle diuretics	Furosemide	C	None reported	Possible association with neonatal PDA and sensorineural hearing loss	Maternal electrolyte monitoring	No
Potassium- sparing diuretics	Spironolactone (not recommended)	C	None reported			Yes (probably safe; (not recommended)
Anticoagulants
Heparin (including LMWHs)	Heparin Enoxaparin Dalteparin	C	None reported	Possible maternal osteoporosis, heparin-induced thrombocytopenia, and bleeding	Maternal platelet and coagulation status monitoring (as appropriate)	Yes
Vitamin K antagonists	Warfarin	X	Fetal warfarin syndrome (craniofacial and skeletal anomalies) with exposure in weeks 6–9; fetal intracranial hemorrhage in second and third trimesters		Comprehensive fetal sonography at 18–20 wk of gestation	Yes	Teratogenicity appears to be related to doses >5 mg daily
Antiplatelet agents	Aspirin (full dose) Clopidogrel	D (third trimester) B	None reported	Premature closure of fetal ductus arteriosus at ≥32 wk of gestation	Empirical recommendation to discontinue clopidogrel 7 d in advance of anticipated delivery	Unknown (AAP) No (WHO)	Low-dose aspirin (≤100 mg/d) does not appear to have deleterious effects
Direct thrombin inhibitors	Argatroban	B	None reported			Unknown
Indirect factor Xa inhibitors	Fondaparinux	B	None reported	Possible neonatal factor Xa inhibition		Unknown
Direct factor Xa inhibitors	Rivaroxaban	C	None reported	Theoretical risk of maternal hemorrhage		Unknown
Fibrinolytics	Streptokinase	C	None reported			Unknown
Inotropic/vasopressors
Cardiac glycosides	Digoxin Digitalis	C	None reported			Yes
Adrenergic agents	Epinephrine Norepinephrine Phenylephrine Dopamine Isoproterenol	C	Possible association with gastroschisis or hemifacial microsomia (phenylephrine)			Probably safe (dopamine) Unknown (epinephrine, norepinephrine, phenylephrine, isoproterenol)
Antiarrhythmics
β-Blockers	Metoprolol Propranolol Carvedilol Atenolol	C C C D	None reported	Association with fetal growth restriction in second and third trimesters (atenolol, propranolol), neonatal bradycardia (esmolol, nadolol)	Consider serial fetal sonography to assess interval fetal growth in second and third trimesters	Except for atenolol, probably safe
Class 1A	Quinidine Procainamide	C C	None reported			Yes
Class 1C	Flecainide	C	None reported			Yes
Class III	Sotalol Amiodarone	B D	None reported	Thyroid dysfunction		No No WHO)/ (AAP Yes)
Purine nucleosides	Adenosine	C	None reported			Unknown
AAP indicates American Academy of Pediatrics; ACE, angiotensin-converting enzyme; FDA, US Food and Drug Administration; LMWH, low-molecular- weight heparin; PDA, patent ductus arteriosus; and WHO, World Health Organisation.

Assessment and Management Women with Complex Congenital Heart Disease

Preconception maternal - fetal risk assessment, Complete history and physical exam, Prior health/surgical records obtained and reviewed, Baseline 12 lead electrocardiogram, echocardiogram, laboratory studies, Cardiopulmonary exercise tolerance and functional class, Medical or surgical repair as indicated, Genetic referral for patients with a heritable cardiac lesion, Discuss discontinuation of teratogenic drugs prior to conception, Appropriate contraception provided until pregnancy desired

Preconception Assessment upon confirmation of pregnancy, Ensure teratogenic medications: ACE, ARB, have been discontinued; If pregnancy was unplanned conduct full maternal-fetal risk assessment and diagnostic evaluation; discuss plan of care (POC) including where to be delivered.

Pregnancy in patient who is clinically stable; Joint management by ACHD and MFM team; establish POC for patients geographically distant from tertiary center; Regular cardiac and OB follow-up; Repeat echocardiogram per trimester as indicated; Fetal echocardiogram 18-22 weeks

Pregnancy in patient with severe ventricular dysfunction (NYHA III or IV), cyanosis, pulmonary hypertension: Discuss maternal-fetal morbidity and mortality risk and outline plan of care including possible need for early hospitalisation. Joint decision regarding pregnancy by patient, family cardiologist, MFM team

Written delivery plan prepared and distributed to team by 28-32 weeks; Vaginal delivery with epidural preferred; C-section reserved for obstetric reasons; Indication of labor as indicated ~39 weeks; Antibiotic prophylaxis as indicated

Terminate Pregnancy; Appropriate contraception or starilization

Joint management by experienced MFM and ACHD teams at tertiary center; Multidisciplinary conference after 26 weeks to discuss L&D management; Close team follow-up increasing as indicated by clinical status; Delivery date determined by clinical status; C-section may be indicated

ACE indicates angiotensin-converting enzyme; ACHD, adult congenital heart disease; ARB, angiotensin receptor blocker; L&D, labor and delivery; MFM, maternal-fetal medicine; NYHA, New York Heart Associa- tion; OB, obstetrics; and POC, plan of care.