Case Report: A Pregnant Woman with Left Ventricular Noncompaction-associated Cardiomyopathy
Key findings
- A 27-year-old woman with a history of familial, left ventricular noncompaction-associated cardiopathy was referred to Massachusetts General Hospital at 20 weeks' gestation for a cardio-obstetrics consultation because of palpitations
- Transthoracic echocardiogram at 22 weeks' gestation demonstrated a left ventricular ejection fraction of 47%
- The patient was managed with beta-blockers, low-dose aspirin and eventually diuretic therapy after she developed clinical heart failure
- Labor was induced at 37 weeks' gestation with central venous pressure monitoring and cardiac anesthesia present; this culminated in an uncomplicated vaginal delivery
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Many pregnant women with preexisting cardiovascular disease can benefit from consulting a specialized cardio-obstetrical team for prenatal and peripartum care, as well as postpartum risk reduction.
In JACC: Case Reports, Nandita S. Scott, MD, co-director of the Corrigan Women's Heart Health Program and co-director of the Cardiovascular Disease and Pregnancy Service at Massachusetts General Hospital, and colleagues report on care for a 27-year-old pregnant woman with familial, left ventricular noncompaction (LVNC)-associated cardiomyopathy.
Case History
The patient was referred to Mass General at 20 weeks' gestation for cardio-obstetrics consultation because she was having palpitations. She had a history of a spontaneous miscarriage at eight weeks of gestation.
The patient had been diagnosed at age 8 with familial, dilated cardiomyopathy, later phenotyped as LVNC-associated cardiomyopathy. This variant is characterized by the prominence of LV trabeculations, deep intertrabecular recesses and a thin, compacted layer of the myocardium.
The patient had not been counseled about the risks of pregnancy associated with her cardiomyopathy. Pregnant women with preexisting dilated cardiomyopathies have major risks of complications including heart failure, arrhythmias, thromboembolism and irreversible deterioration in LV function postpartum. Therefore expert consensus guidelines recommend preconception counseling.
Prior to conception the patient had used beta-blockers and angiotensin-converting enzyme (ACE) inhibitors and maintained a left ventricular ejection fraction (LVEF) of 52%. The ACE inhibitors had been discontinued preconception. ACE inhibitors, angiotensin receptor blockers, mineralocorticoid receptor antagonists and ivabradine are contraindicated antepartum because they are teratogenic.
Investigations
- Ambulatory ECG monitoring demonstrated rare premature ventricular contractions without sustained supra or ventricular tachyarrhythmias
- Transthoracic echocardiogram (TTE) at 22 weeks' gestation demonstrated LVEF of 47% and a ratio of noncompacted to compacted (NC/C) myocardium of 2.6 at end-systole
- Cardiac MRI performed before conception had demonstrated an LVEF of 49% and an NC/C myocardium ratio of >2.3
Management
Beta-blocker therapy was continued throughout pregnancy. It should be noted that most beta-blockers, particularly atenolol, can cause fetal growth restriction. There are no guidelines for pharmacological care of LVNC-associated cardiomyopathy during pregnancy.
Low-dose aspirin was also started. The optimal strategy for managing thromboembolic risk in pregnant women with LVNC-associated cardiomyopathies is unclear, and decision-making about treatment choice should be shared with the patient.
Diuretic therapy was initiated after the patient developed progressive dyspnea and clinical heart failure.
Genetic Counseling and Testing
The patient was referred for genetic counseling and genetic testing of the fetus. Most genes linked to LVNC cardiomyopathy are associated with an autosomal dominant pattern of inheritance, which was the case in the patient's family. Her child did not carry the genetic variant.
Delivery
Repeat TTE at 36 weeks demonstrated a decline in LVEF to 35% with progressive LV dilation. The decision was made to induce labor at 37 weeks' gestation with central venous pressure monitoring with cardiac anesthesia present. This culminated in an uncomplicated vaginal delivery.
The mode of delivery in the setting of LV dysfunction should be discussed by the multidisciplinary cardio-obstetrical team and the patient. In patients with stable heart failure, vaginal delivery is preferred, with cesarean delivery usually reserved for obstetrical indications.
Postnatal Risk Reduction
Postpartum TTE, conducted once the patient was back on both ACE inhibitor and beta-blocker therapy, demonstrated an LVEF of 39% and a decrease in LV chamber size. The patient was counseled about the risks of another pregnancy. Using the rating scale developed in the CARPREG (Cardiac Disease in Pregnancy) II study, her risk of an adverse cardiac event during a subsequent pregnancy was 41%. At the time of this report, the patient was planning a gestational carrier pregnancy.
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Refer a patient to the Corrigan Minehan Heart Center at Mass General