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[edit | edit source]

### Modified Who Classification of Maternal Cardiovascular Risk[edit | edit source]

Who Pregnancy Risk Category | Risk Description | Maternal Risk Factors

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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[edit | edit source]

| Common Examples | FDA Pregnancy Category | Teratogenic risks (First Trimester) | Other Pregnancy Concerns (Second and Third Trimesters) | Suggested Evaluation | Lactation | Notes

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Antihypertensives
β-Blockers | Metoprolol Propranolol Carvedilol Atenolol | C

| 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

| 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

| 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)

| 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

| 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

| None reported |  |  | Yes |
Class 1C | Flecainide | C | None reported |  |  | Yes |
Class III | Sotalol Amiodarone | B

| 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|

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| 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.