**7.11 Peripartum cardiomyopathy**

The syndrome is a rare disorder of pregnancy. It was recognized in 1937, as a distinct clinical entity (Gouley et al., 1937). Currently, the etiology of peripartum cardiomyopathy (PPCM) remains unclear. However, there is compelling data from animal and human studies suggesting that PPCM is actually a type of myocarditis arising from an infectious, autoimmune, or idiopathic etiology. The relationship between pregnancy and viral myocarditis was first published in 1968 (Farber & Glasgow, 1970). Endomyocardial biopsies in women with PPCM have demonstrated myocarditis in many patients. The highest incidence of myocarditis reported in PPCM was 76 percent (Midei et al., 1990), however much lower incidence was reported (8.8 percent), which found to be comparable to an age and sex matched control population undergoing transplantation for idiopathic dilated cardiomyopathy (9.1 percent), (Rizeq et al., 1994). Viral genomes of parvovirus B19, human herpes virus 6, Epstein–Barr virus, and human cytomegalovirus revealed in endomyocardial biopsy specimens from patients with PPCM (Bultmann et al., 2005). Other reported data linked with Chlamydial infection (Cenac et al., 2003).

cardiomyopathy yet with new ventricular arrhythmias or second-degree or third degree heart block or who do not have a response to optimal care are more likely to have cardiac sarcoidosis (Yazaki et al., 1998). Cardiac symptoms were reported in 101 patients, when cardiac sarcoidosis was diagnosed in 84 percent compared to 4 percent in asymptomatic patients (Smedema et al., 2005). Endomyocardial biopsy shows characteristic noncaseating granulomas. However, the diagnosis can also be inferred if there is a tissue diagnosis of sarcoidosis from an extracardiac source in the presence of a cardiomyopathy of unknown

The electrocardiographic abnormalities found in nearly 70 percent of patients with sarcoidosis (Chapelon-Abric et al., 2004). Cardiac involvement with sarcoidosis may produce clinical symptoms and electrocardiographic findings simulating myocardial infarction. Conduction abnormalities in form of first-degree heart block due to disease of the atrioventricular node or bundle of His, and various types of intraventricular conduction defects, are common among patients with cardiac sarcoidosis (Chapelon-Abric et al., 2004). These lesions may initially be silent, but can progress to complete heart block and cause syncope (Yoshida et al., 1997). Sustained or nonsustained ventricular tachycardia and ventricular premature beats are the second most common presentation of cardiac sarcoidosis; electrocardiography reveals ventricular arrhythmias in as many as 22 percent of patients with sarcoidosis (Sekiguchi et al., 1980). Supraventricular arrhythmias are infrequent. Sudden death due to ventricular tachyarrhythmias or conduction block accounts for 25 to 65 percent of deaths due to cardiac sarcoidosis, however, sudden death can occur in the absence of a previous cardiac event (Reuhl et al., 1997; Soejima & Yada, 2009; Yazaki et al., 2001). Both systolic and diastolic heart failure can occur. Left ventricular aneurysms develop in patients with extensive involvement of the myocardium. Mitral incompetence may occur with cardiac sarcoidosis due to associated systolic dysfunction and left ventricular dilation or due to papillary muscle involvement by sarcoid granulomas (Sato et al., 2008). Tricuspid regurgitation with atrioventricular block secondary to infiltration of tricuspid valves and conduction system by sarcoid granulomas has been reported as well (Goyal & Aragam, 2006). A left atrial granulomatous mass resembling myxoma has been

The syndrome is a rare disorder of pregnancy. It was recognized in 1937, as a distinct clinical entity (Gouley et al., 1937). Currently, the etiology of peripartum cardiomyopathy (PPCM) remains unclear. However, there is compelling data from animal and human studies suggesting that PPCM is actually a type of myocarditis arising from an infectious, autoimmune, or idiopathic etiology. The relationship between pregnancy and viral myocarditis was first published in 1968 (Farber & Glasgow, 1970). Endomyocardial biopsies in women with PPCM have demonstrated myocarditis in many patients. The highest incidence of myocarditis reported in PPCM was 76 percent (Midei et al., 1990), however much lower incidence was reported (8.8 percent), which found to be comparable to an age and sex matched control population undergoing transplantation for idiopathic dilated cardiomyopathy (9.1 percent), (Rizeq et al., 1994). Viral genomes of parvovirus B19, human herpes virus 6, Epstein–Barr virus, and human cytomegalovirus revealed in endomyocardial biopsy specimens from patients with PPCM (Bultmann et al., 2005). Other reported data

origin.

reported too (Abrishami et al., 2004).

**7.11 Peripartum cardiomyopathy** 

linked with Chlamydial infection (Cenac et al., 2003).

Women present with heart failure during the peripartum period and become manifested in the last month of pregnancy or within 5 months of the delivery without apparent etiology for the heart failure can be found. The clinical scenario is challenging because many normal women in the last month of a normal pregnancy experience dyspnea, fatigue and pedal edema, symptoms can mimic early congestive cardiac failure. Physical examination can be significant for signs of right and left heart failure. Symptoms and signs that should raise the suspicion of heart failure include paroxysmal nocturnal dyspnea, chest pain, nocturnal cough, new regurgitant murmurs, pulmonary crackles, elevated jugular venous pressure and hepatomegaly. The electrocardiogram usually demonstrates a normal sinus or sinus tachycardia rhythm, but frequent ectopy and other atrial arrhythmias may also be present. Left ventricular hypertrophy, inverted T waves, Q waves, and nonspecific ST-T changes have also been reported (Brown & Bertolet, 1998). Recurrence in a subsequent pregnancy has been reported. However, significant improvement occurs in up to 50 percent of affected women; others are left with a progressive dilated cardiomyopathy.
