Peripartum cardiomyopathy

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 five months post-partum.

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.

The etiology of postpartum cardiomyopathy is unknown.

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Prevalence and Risk Factors

PPCM is a diagnosis of exclusion, wherein patients have no prior history of heart disease and there are no other causes of heart failure.

According to PPCM Investigator James D. Fett, MD, "In the United States, the estimated incidence is between 1 in 2000 to 4000 live births. No precise figure is available because there are no population-based PPCM registries in the USA. In some countries the incidence seems to be higher, such as South African Bantus with an estimated 1 case per 1000 live births, and Haiti with a calculated incidence of 1 case per 350 live births" [1].

The incidence of peripartum cardiomyopathy is increased in women over the age of 30, in twin pregnancies, in multiparous women, in women with gestational hypertension, those who have received tocolytic therapy, and in african americans; however, PPCM also inflicts women who do not have any of these risk factors.

Echocardiogram is the definitive diagnostic tool of PPCM.

Clinical Features (History)

Pregnancy itself brings about some features that suggest cardiac insufficiency. Symptoms such as dyspnoea, dizziness, orthopnoea and decreased exercise tolerance are often normal findings in pregnancy.

Moreover, the normal cardiac physiology changes dramatically in the gravid female. Blood volume increases progressively from 6-8 weeks gestation (pregnancy) and reaches a maximum at approximately 32-34 weeks with little change thereafter. During the first trimester cardiac output is 30-40% higher than in the non-pregnant state; there is also an approximately 35% increase in stroke volume, a 15% increase in HR and a steady decrease in vascular resistance.

Since this is excellent cover for a developing condition, often there are no extreme or notable symptoms until several days post-partum when the most notable symptoms become pulmonary edema with resultant breathing difficulties(dysnopea), serious energy depletion performing simple tasks such as walking, standing or even sitting up for extended periods, and sudden cardiac arrest.

If these conditions appear after a woman has been discharged from clinical care the possibility of mortality is greatly increased.

The dyspnoea is described usually by women as the inability to take a deep breath to get enough air into her lungs. It is thought that the hormonally mediated (progesterone) hyperventilation seen in pregnancy is the cause.

In PPCM the symptoms secondary to acute onset of heart failure seen are similar to patients with systolic dysfunction who are not pregnant. These symptoms include cough, orthopnoea, paroxysmal nocturnal dyspnoea, fatigue, palpitations, haemoptysis and chest pain. These symptoms mimic many normal pregnant women who cannot tolerate lying flat, have significant pedal edema, complain of shortness of breath and dyspnea but have no heart disease.

The challenge facing physicians is to differentiate between these two groups and diagnose women with PPCM sooner than later as early intervention has shown benefit and may save lives.

Prognosis

The most recent studies indicate that with newer conventional treatment, the survival rate is very high at 97% or better^1,2,3. Most PPCM patients improve with treatment. Almost two-thirds of patients experience complete recovery (ejection fraction greater than 50%). Most fully recovered patients are eventually able to discontinue medications and have normal life expectancy.

In the recent past, PPCM patients, whether recovered or not, have been almost universally discouraged from considering another pregnancy. However, for the recovered patient with an EF >50%, subsequent pregnancy may still be an option. An algorithim for subsequent PPCM pregnancy provided by Dr. James D. Fett [2] asserts that for women who have recovered full heart function, the risk of recurrent heart failure for one subsequent pregnancy is equal to or less than 10% and the risk of death is less than 2%.

The New York Peripartum Cardiomyopathy Study Group is an ongoing registry seeking to answer many of the questions left unanswered because of the rarity of the disease. For more information about the study, one can visit their website at www.amothersheart.org [3].

Treatment

Treatment for the disease is similar to treatment for congestive heart failure. Conventional heart failure treatment includes use of diuretics, beta blockers, and angiotensin-converting enzyme inhibitors.³ Delivery is the recommended overall treatment to decrease the volume load, improve ventricular function and simplify the medical management of these patients.

References

1. Felker GM, Jaeger CJ, Klodas E, Thiemann DR, Hare JM, Hruban RH, Kasper EK, Baughman KL. Myocarditis and long-term survival in peripartum cardiomyopathy. American Heart Journal 2000;140:785-91.

2. Amos AM, Jaber WA, Russell SD. Improved outcomes in peripartum cardiomyopathy with contemporary treatments. American Heart Journal 2006; 152:509-13.

3. McNamara. Left ventricular recovery in peripartum cardiomyopathy: Impact of beta-blockade. Circulation 2007;116, October 16, Supplement II, page 551, Abstract #2500.

4. Sliwa K, Fett JD, Elkayam U. Seminar: Peripartum cardiomyopathy. Lancet 2006;368:687-93.

5. Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, Ansari AA, Baughman KL. Peripartum cardiomyopathy. National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) Workshop recommendations and review. JAMA 2000;283:1183-88.

6. Elkayam U, Padmini P, Tummala P, Rao K, Akhter MW, Karaalp IS, Wani OR, Hameed A, gviazda I, Shotan A. Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy. New England Journal Medicine 2001;344:1567-71.

7. Ansari AA, Fett JD, Carraway RD, Mayne AE, Onlamoon M, Sundstrom JB. Autoimmune mechanisms as the basis for human peripartum cardiomyopathy. Clinical Review Allergy Immunology 2002;23:289-312.

8. Fett JD, Christie LG, Carraway RD, Sundstrom JB, Ansari AA, Murphy JG. Unrecognized peripartum cardiomyopathy in Haitian women. International Journal Gynaecology Obstetrics 2005;90:161-6.

9. Elkayam U, Akhter MW, Singh HS, Khan S, Bitar F, Hameed A, Shotan A.Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 2005;111:2050-55.

10. Warraich RS, Fett JD, Damasceno A, Carraway RD, Sundrom JB, Arif J,Essop R, Ansari AA,Yacoub MH, Sliwa K. Impact of Pregnancy related heart failure on humoral immunity: clinical relevance of G3-subclass immunoglobulinss in peripartum Cardiomyopathy. American Heart Journal 2005;150:263-9.

11. Sliwa K, Forster O, Libhaber E, Fett J, Sundstrom JB, Hilfiker-Kleiner D,Ansari AA. Peripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectively studied patients. European Heart Journal. 2006;27(4):441-6.

12. Ro, Angela; Frishman, William. Cardiology in Review 2006;14: 35–42.