Cardiac disease in pregnancy and peripartum cardiomyopathy

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Pathophysiology

Epidemiology and Demographics

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Diagnosis

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Catheterization:

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Special Scenarios:

I. Pre-existing Cardiac Disease:
Congenital Heart Disease
Repaired Congenital Heart Disease
Pulmonary Hypertension
Rheumatic Heart Disease
Connective Tissue Disorders
II. Valvular Heart Disease:
Mitral Stenosis
Mitral Regurgitation
Aortic Insufficiency
Aortic Stenosis
Mechanical Prosthetic Valves
Tissue Prosthetic Valves
III. Cardiomyopathy:
Dilated Cardiomyopathy
Hypertrophic Cardiomyopathy
Peripartum Cardiomyopathy
IV. Cardiac diseases that may develop During Pregnancy:
Arrhythmias
Acute Myocardial Infarction
Hypertension

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Anjan K. Chakrabarti, M.D. [2]

Synonyms and Keywords: PPCM; PPCMP

Overview

Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting between the last month of gestation and up to six months post-partum. The etiology of postpartum cardiomyopathy is unknown. Reported prevalence of postpartum cardiomyopathy in United States is estimated to be 1 case per 1300-15,000 live births. Treatment for the disease is similar to treatment for congestive heart failure. Delivery is the recommeded overall treatment to decrease the volume load, improve ventricular function and simplify the medical management of these patients.

Definition

Peripartum cardiomyopathy is defined as:

  • Ejection fraction <45% and/or
  • Fractional shortening <30%
  • End-diastolic dimension >2.7 cm/m2 BSA (body surface area)

Pathophysiology

The etiology of postpartum cardiomyopathy is largely unknown. It has been postulated that a defective antioxidant mechanism may contribute. There are elevated levels of cathepsin D and an increase in total prolactin and angiostatic 16kDa prolactin. These molecules promote:

The potential role of prolactin forms the molecular basis of treatment with bromocriptine.

As with other forms of dilated cardiomyopathy, PPCM involves decrease of the left ventricular ejection fraction with associated congestive heart failure and increased risk of atrial and ventricular arrhythmias and even sudden cardiac death.

Differentiating Peripartum Cardiomyopathy from Other Diseases

Epidemiology and Demographics

  • Estimates of incidence 1/1,300-15,000.

Risk Factors

PPCM is more common among women with:

  • Prior PPCM
  • Multiple pregnancies
  • African decent, Haitian descent
  • History of toxemia
  • Long-term tocolytic use
  • Age >30
  • Twin Pregnancy

Natural History, Complications and Prognosis

  • Some patients will have a relapse of PPCM after a partial or full recovery.
  • The course of peripartum CMP is variable.
  • PPCM is a leading cause of pregnancy related death in the United States.
  • Mortality 25-50% (half deaths in first 3 months).
  • 50% improve within 6 months.
  • 20% to 40% of patients normalize their LVEF.
  • If the LVEF remains poor at 6 months, then mortality is increased.
  • PPCM is a risk factor for recurrence with subsequent pregnancies.
  • Favorable outcomes with cardiac transplantation.

Diagnosis

History and Symptoms

Signs and symptoms are similar to those of normal pregnancy

Treatment

Medical Therapy

  • Delivery is the recommeded overall treatment to decrease the volume load, improve ventricular function and simplify the medical management of these patients.

Pharmacotherapy

Pharmacotherapy should be discontinued or tapered gradually based upon repeated echocardiographic analyses. Some patients will have a relapse of PPCM after a partial or full recovery.

Surgery

References


Cardiology