An unusual case of peripartum cardiomyopathy manifesting with multiple thrombo-embolic phenomena
© Ibebuogu et al; licensee BioMed Central Ltd. 2007
Received: 03 September 2007
Accepted: 29 October 2007
Published: 29 October 2007
Peripartum cardiomyopathy (PPCM) is a rare form of heart failure with a reported incidence of 1 per 3000 to 1 per 4000 live births and a fatality rate of 20%–50%. Onset is usually between the last month of pregnancy and up to 5 months postpartum in previously healthy women. Although viral, autoimmune and idiopathic factors may be contributory, its etiology remains unknown. PPCM initially presents with signs and symptoms of congestive heart failure and rarely with thrombo-embolic complications. We report an unusual case of PPCM in a previously healthy postpartum woman who presented with an acute abdomen due to unrecognized thromboemboli of the abdominal organs. This case illustrates that abdominal pain in PPCM may not always result from hepatic congestion as previously reported, but may occur as a result of thromboemboli to abdominal organs. Further research is needed to determine the true incidence of thromboemboli in PPCM.
Peripartum cardiomyopathy (PPCM) is a rare form of heart failure of unknown cause with a reported incidence of 1 per 3000 to 1 per 4000 live births. Onset is usually between the last month of pregnancy and up to 5 months postpartum in previously healthy women, with a reported fatality rate of 20%–50%. Although viral, autoimmune and idiopathic factors may be contributory, its etiology remains unknown. PPCM usually presents initially with signs and symptoms of heart failure and rarely with thrombo-embolic complications. We report an unusual case of PPCM in a previously healthy woman who presented with an acute abdomen due to multiple thromboemboli.
Peripartum cardiomyopathy (PPCM) is a rare form of dilated cardiomyopathy that is associated with a high maternal morbidity and mortality, reportedly accounting for 4% of maternal deaths in the United States [1, 2]. It is defined according to the following four criteria: 1) Development of cardiac failure in the last month of pregnancy or within five months of delivery; 2) absence of identifiable cause for the heart failure; 3) absence of recognizable heart disease prior to the last month of pregnancy; and 4) left ventricular systolic dysfunction demonstrated by echocardiographic criteria [1, 3]. The exact etiology remains unknown. The incidence of PPCM ranges from 1:3000 to 1:4000 live births and it affects 1000 to 1300 women in the United States each year . The mortality rate is reportedly 20% to 50%, and death occurs as a result of progressive heart failure, arrhythmias and thromboembolism . PPCM presents initially with signs and symptoms of heart failure and rarely with thromboembolic complications [3, 6–8]. The occurrence of thromboembolism in PPCM may be due to the hypercoagulable state of pregnancy and the left ventricular dysfunction which causes a relative blood stasis . This warrants the initiation of anticoagulation therapy in the presence of severe left ventricular dysfunction. Upper abdominal discomfort occurs in approximately half of the patients diagnosed with PPCM as a result of an enlarged congested liver . Our patient's initial presentation was with severe abdominal pain. After she was diagnosed with PPCM, her abdominal pain was initially ascribed to hepatic enlargement and congestion due to congestive heart failure. Due to worsening of her abdominal pain, despite resolution of her congestive heart failure symptoms, and normal right heart pressures on cardiac catheterization, a computed tomography contrast scan of the abdomen was ordered. This revealed multiple thromboemboli of the heart, kidneys, and common iliac and superficial femoral arteries. Based on the nature of her symptoms, and the occurrence of multiple emboli in the abdominal organs, ischemia from thromboemboli was the determined cause of her severe abdominal pain. Our patient was started on anticoagulation at the onset due to the presence of an intracardiac thrombus with severe systolic dysfunction on echocardiography. This played a role in the termination of the thromboembolic process and the resolution of her abdominal symptoms. The current consensus in the management of PPCM is to initiate anticoagulation therapy in the presence of severe left ventricular function (LVEF ≤ 35%) . However, abdominal pain in the absence of congestive heart failure symptoms, may indicate the presence of thromboemboli of the abdominal organs. This may be a further indication for anticoagulation. Early diagnosis and treatment of PPCM are essential for a favorable outcome and poor prognostic factors include high parity, twin gestation, age greater than 30 years, and a late onset of symptoms after delivery . Our patient is multiparous and her clinical presentation occurred 5 months postpartum. A high clinical index of suspicion, cardiomegaly and a severe left ventricular dysfunction prompted accurate diagnosis and a successful therapy. In patients with PPCM the return of the left ventricle size and function to normal in the first 5 months of the postpartum period is a good prognostic factor. This usually occurs in 50% of patients with PPCM. Our patient's left ventricular dysfunction persisted greater than 6 months after hospital discharge, and she is currently awaiting heart transplant.
Patients with peripartum cardiomyopathy (PPCM) may rarely be predisposed to thromboembolic phenomenon due to blood stasis resulting from the hypercoagulable state of pregnancy and the depressed left ventricular systolic function typical of this disease. Abdominal pain may not always indicate the presence of hepatic enlargement and congestion as previously believed, but may occur as a result of non-occlusive thromboemboli of the bowel or other abdominal organs as reported in our patient. This may be a further indication for early initiation of anticoagulation to prevent a potential adverse outcome from bowel or other abdominal organ ischemia. Further research is needed to determine the true incidence of thromboemboli in PPCM.
PPCM – Peripartum Cardiomyopathy
CT – Computed tomography
ED – Emergency department.
Written consent was obtained from the patient and their relatives for the publication of this report.
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