Peripartum cardiomyopathy coexistent with human immunodeficiency virus: a substantial obstetric jeopardy.

Peripartum cardiomyopathy (PPCM) is a rare cause of pregnancy-related heart failure, which affects a woman during the last months of pregnancy or first months of parturition. Its etiopathogenesis is still unclear. Coexistence of PPCM with human immunodeficiency virus (HIV) has been scarcely analyzed. A low CD4 count is proposed to be one of the predictors of dilated cardiomyopathy in HIV. Here, a pregnant woman with HIV presented with signs of congestive heart failure for the first time during her last trimester. Echocardiography revealed a dilated cardiomyopathy with ejection fraction of 34% which proved the diagnosis of PPCM. She underwent cesarean section for impending previous scar rupture. Her status deteriorated subsequently in spite of all efforts and she succumbed due to ventricular tachycardia. This case necessitates an awareness regarding coexistence of HIV with PPCM and dreaded clinical sequences. Patients suffering from HIV should be treated well and their CD4 count should be improved before conception to avoid such complications in pregnancy.

INTRODUCTION

Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy with severe left ventricular (LV) dysfunction leading to heart failure during the last month of pregnancy or within 5 months of delivery without any determinable etiology for the cardiac failure and also absence of demonstrable heart disease prior to the last month of pregnancy.[1] Its incidence is variable, ranging from 1 in 1300 to 1 in 15,000 pregnancies, and is more prevalent in Haiti and certain parts of Africa.[1] Risk factors include multiparity, advanced maternal age, multiple pregnancies, pre-eclampsia, chronic hypertension, smoking, alcoholism, malnutrition, and long-term tocolysis.[1] Although the etiopathogenesis of PPCM is as yet unknown, in certain cases it is being associated with various viral infections like coxackievirus group B and Epstein-Barr virus.[2] Human immunodeficiency virus (HIV) infection is also increasingly being recognized as an important cause of dilated cardiomyopathy (DCM),[3] but coexistence with PPCM is as yet infrequently reported.[4] A thorough Medline search revealed only a single article depicting PPCM with HIV from Africa, which describes 34% HIV co-infected cases in 80 consecutive PPCM women. As such, articles are yet to be reported from the Indian subcontinent. We present a rare case of PPCM with HIV to draw attention to its clinical presentation and sequelae, and thus create an awareness regarding the outcome of this dreaded combination.

CASE REPORT

A 31-year-old second gravidae with history of previous section attended the emergency OPD with chief complaint of shortness of breath for the last 4 days. She did not report any prior attacks of respiratory distress either in the present or previous pregnancy.

On examination, the woman was afebrile and orthopneic with mild pallor. She had a respiratory rate of 34/min, and tachycardia along with a normal blood pressure. Obstetric examination revealed a term-sized live fetus in longitudinal lie with cephalic presentation. There was scar tenderness, and vaginal examination showed a closed cervix. On auscultation, mild bibasilar crepitations and a loud S3 were audible.

In view of the obstetric indication and impending LV failure, cesarean section was carried out with diuretics. A healthy boy weighing 3.405 kg with good Apgar score was delivered. On the first puerperal day, her condition further deteriorated, necessitating transfer to intensive coronary care unit (ICCU) for better management. Laboratory investigations at admission revealed hemoglobin level at 8.11 gm/dl, total count 15,700/mm3, platelet count 90,000/mm3, normal blood glucose and liver function test. Blood urea was 67 mg/dl and serum creatinine was 1.6 mg/dl.

Electrocardiography (ECG) showed atrial fibrillation. Chest X-ray on the first puerperal day had features of cardiomegaly and pulmonary congestion. Echocardiography findings were consistent with DCM with ejection fraction (EF) of 34%, grade II MR, and features of congestive cardiac failure. Surprisingly, her HIV status showed a high seropositivity by standard diagnostic technique. CD4 count was 250.

There was progressive increase in respiratory distress due to worsening heart failure. Supportive management included propped up position, moist O2 inhalation, inotropic drugs, broad-spectrum antibiotics, diuretics, Angiotensin converting enzyme (ACE) inhibitor, digoxin, and thromboprophylactics. In spite of these efforts, she succumbed due to ventricular tachycardia on the second postoperative day.

DISCUSSION

PPCM is an idiopathic DCM cardiomyopathy with heart failure secondary to LV dysfunction occurring in the last month of gestation or in 5 months of delivery, where no other cause can be found. The echocardiographic criteria are: EF <45%, fractional shortening <30%, or both, with or without left ventricular end diastolic dimension (LVEDD) >2.7cm/m 2. Morbidities include LV heart failure, persistent left ventricular dysfunction (LVEF <45% after 6 months of initial diagnosis of PPCM), increased risk of thromboembolism, preterm labor and its neonatal consequences, etc.[5] It is a life-threatening cardiac abnormality with mortality rates between 18% and 56%, and half the deaths happen in the first month after delivery.[1] Predictors of the poor prognosis of PPCM are EF <25%, left ventricular end systolic dimension (LVESD) >5.5 cm, QRS time in ECG <120 ms, and also a subsequent pregnancy.[56] Thus, future pregnancies are usually discouraged in PPCM patients due to its high mortality state.[5]

Multiple etiopathogenetic theories have been cited for PPCM. One of the recent hypotheses suggests that oxidative stress, the prolactin cleaving protease cathepsin D, and high level of prolactin are usually responsible for development for this disease.[7] Again, authors like Barbaro, et al. found 8% incidence of DCM in 952 asymptomatic HIV-positive patients.[3] Interestingly, DCM was higher in patients with CD4 count <400 cells/mm3 and in those who had received zidovudine therapy. The pathogenesis was mainly related to in situ hybridization and myocarditis induced by autoimmune process of HIV. Regarding the coexistence of PPCM and HIV, only one article has been published till date. In that, Silwa, et al., while researching the long-term outcome of PPCM in population with high seropositivity for HIV, observed 34% co-infection with HIV in a total of 80 PPCM patients.[4] However, they stated that the continuous high mortality of PPCM patients occurring beyond 6 months was independent of HIV infection and subsequent pregnancy. So, co-infection with HIV can be counted as another predictor of unfavorable prognosis of PPCM.

Our patient had an irregular antenatal follow-up and was diagnosed to have HIV with very poor CD4 count for the first time during admission only. She had never received Antiretroviral therapy (ART). Except previous pregnancy, she had no other risk factors for PPCM. A poor EF of <34% along with co-infection with HIV and poor CD4 count might be the major predictors of her mortality. Treatment options include diuretics, beta blockers, angiotensin converting enzyme inhibitors especially in the postnatal period, etc., Anticoagulation has got importance in preventing thromboembolic phenomenon. As prolactin has been implicated in pathogenesis, blockade of this pathway by bromocriptine therapy is becoming a novel approach currently.[8] Avoidance of breastfeeding has been also seen to be beneficial.

This case is unique and rare, depicting clinical sequences as well as outcome. The patients coming for antenatal visits with breathlessness complaint must undergo ECG and later on echocardiography, as and when required. Patients with associated risk factors like pregnancy induced hypertension (PIH), multiple pregnancies, co-infection with HIV, etc., can be dealt with more vigilant check-ups. This clinical awareness is desirable for the patients and clinician as well. Early diagnosis, treatment, and multidisciplinary approach may avoid substantial obstetric catastrophes.