An intriguing challenge of unveiling the unexpected: Peripartum cardiomyopathy

Harish M.M¹, Alankrita Agarwal², Chaitra S², Mohammad Abbas³, Gururaj V Gunda³, Shridhar M Gidaganti³, Ashwini N A³, Goura Lokanand³

¹Director-Institute of Critical Care Medicine, Kauvery Hospital, Marathahalli

²Senior Resident, Critical Care Medicine, Kauvery Hospital, Marathahalli

³Consultant, Critical Care Medicine, Kauvery Hospital, Marathahalli

Abstract

A 21-year-old female without any prior co-morbidities underwent emergency Lower Segment Cesarean Section (LSCS) due to meconium-stained amniotic fluid. Postoperatively, she developed severe left ventricular systolic dysfunction (LVEF – 25%) and cardiogenic shock, necessitating high-dose multi-vasopressor support. Her condition was further complicated by severe pulmonary edema requiring mechanical ventilation and multiorgan dysfunction.

She was referred to our center for possible extracorporeal membrane oxygenation (ECMO) support. Following aggressive management; including optimal inotropic support, treatment of acute pulmonary edema, ischemic hepatitis, and acute kidney injury, the patient showed gradual but significant improvement. She was successfully extubated, weaned off vasopressors, and stabilized hemodynamically. Ultimately, she made a full recovery and was discharged in stable condition.

This case underscores the critical importance of early recognition and individualized management in rare and life-threatening conditions such as peripartum cardiomyopathy.

Introduction

Peripartum cardiomyopathy (PPCM) is a rare and idiopathic form of systolic heart failure that typically occurs in the late stages of pregnancy, most often in the last month or within five months postpartum. Its incidence is estimated at 1 in 3,000 to 4,000 live births, with mortality rates ranging from 20–50%. The condition involves weakening and dilation of the heart muscle, impairing its ability to pump blood effectively. Common symptoms include shortness of breath, fatigue, and peripheral swelling, resembling typical signs of heart failure.

Diagnosis is made after excluding other potential causes, with echocardiography revealing reduced ejection fraction. Identified risk factors include maternal age over 30, African descent, multiple gestations, and preeclampsia. Management generally follows standard heart failure treatment protocols. Recovery outcomes vary, and subsequent pregnancies carry a significantly higher risk of recurrence. Timely diagnosis and appropriate management are essential for improving prognosis.

Case Presentation

A 21-year-old primigravida, with a recent history of emergency LSCS performed on 19/03/2025 at a local hospital in Rayachoti, Andhra Pradesh, presented with complaints of breathlessness, tachycardia (HR 130 bpm), and hypotension (BP 80/50 mmHg) approximately 12 hr postoperatively. Given her hemodynamic instability and signs of respiratory distress, she was intubated and placed on mechanical ventilation with vasopressor support. A transthoracic echocardiogram revealed severe left ventricular dysfunction (Ejection fraction approximately 25%).

In view of her deteriorating condition and the potential need for extracorporeal membrane oxygenation (ECMO), the patient was transferred via ambulance to Kauvery Hospital, Marathahalli. She arrived on 20/03/2025 at 10:00 pm, intubated, with a right femoral central venous catheter in place, and receiving high dose of vasopressor support (Noradrenaline and Vasopressin). Vasopressors were optimized (Started on Dobutamine and Adrenaline supports) given trial for possible recovery before initiation of ECMO. Her Blood investigations revealed AKI with Metabolic acidosis, Acute Liver Injury, which was treated with supportive measures. Patient started responding her blood pressures Improved, pulmonary edema started improving. Vitals showed HR of 80bpm and BP of 90/60 mm Hg with high dose of Noradrenaline @ 10ml/hr and Vasopressin Infusion@2.4ml/hr, Spo2 of 100%.

She was taken on ventilatory support of PRVC Mode with TV 400 ml, PEEP 8 cm H2O, RR 20/min, FiO2 of 80%. Cardiology Reference with 2D echo, Gynaecological Reference, USG Abdomen+Pelvis were taken.

On arrival 2D echo showed Global LV Hypokinesia with regional variation, Severe Left Ventricular Systolic Dysfunction with EF 30%, with Trivial MR/AR. As per the investigations, patient was taken on Inj Meropenem 1 gm 8^th hourly, Inj Polymyxin B 15 lac IU stat followed by 7.5 Lac IU on 20/3/2025.

Patient started holding on to the vitals and so Infusion Noradrenaline, Vasopressin and Dobutamine were stopped. On 21/3/2025 patient was continued with Infusion Fentanyl at 50 microgram/hour with Infusion Furosemide at 2mg/hour. Fever Spikes were up to 101.5-degree Fahrenheit once a day for which tepid sponging was regularly done. Up-trending of liver enzymes was seen. Hepatoprotective measures were taken (Inj N-acetylcysteine, Tab Rifaximin 550 mg 12^th hourly, Syp Duphalac 10ml 8^th hourly).

On 22/3/2025, patient was hemodynamically stable and fully responsive so was extubated with minimal support of Infusion Dobutamine. Gradually AKI and ALI improved. She was continued on diuretics and anti-failure medication (Beta Blockers, ARNI, Bromocriptine).

Severe Transaminitis started downtrending. Blood Culture, Urine Culture and Sputum Culture were sterile. Urine Output started Improving to 60-70 ml/hr. WBC counts started Normalizing with reduction in the anasarca. Procalcitonin Levels were on decreasing trend and Patient was mobilized by 24/3/2025 and so patient was shifted to ward.

Patient was managed symptomatically and kept under observation in ward for 5 days. Repeat 2D-Echo was done on 30/3/2025 which showed significant improvement in the cardiac function with Normal Chamber dimensions, Normal Left Ventricular Systolic function with EF of 58%.

Investigations

USG Abdomen with Pelvis on 21/3/25 – Post Partum Uterus with heterogenous Endometrium with no RPOC.
Transthoracic Echo on 21/3/25 – Normal chamber size with global hypokinesia of LV, Severe LVSD(EF-30%), LVDD, Mild MR/TR with no PAH with Post Partum Cardiomyopathy.
CT Abdomen with Pelvis on 23/3/25– Post LSCS status with post operative changes in pelvis, a small hematoma over the urinary bladder, bulky uterus with scar with bilateral symmetrical moderate pleural effusion.

Lab Parameters

Parameters	21/3/25	22/3/25	23/3/25	24/3/25	25/3/25	26/3/25
HB	11.8	11.8		10.12		8.59
TLC	23880	24100	21810	20530	20410	17340
Bilirubin	1.2		1.1	1.5		1.0
SGOT	526	995	2808	470	85	48
SGPT	271	912	2595	1397	657	258
Albumin	1.6		1.5	1.3		1.3
PT	19.0	14.5	11.6	11.6
Urea	68.48	55.6	44		25
Creatinine	1.04	0.7	0.6		0.5
Procal		10.83			1.36

ABG Analysis

Date	pH	pCO2	pO2	HCO3	Lactate
21/3/25 (11 am)	7.5	34	179	30.4	1.3
21/3/25 (8 pm)	7.5	36	144	30.1	1.2
22/3/25 (5:00 am)	7.5	35	152	27.9	1.4
22/3/25 (on FM)	7.5	37	189	28.9	1.5
23/3/25 (4:00 am)	7.4	39	170	27.7	1.3
24/3/25 (4:00 am)	7.5	40	115	31.2	1.3

Vital Monitoring

Vitals	20/3/25	21/3/25	22/3/25	23/3/25	24/3/25	24/3/25
HR	90/min	100/min	120/min	110/min	100/min	95/min
BP	120/80	100/60	110/70	100/70	110/60	110/70
RR	24/min	28/min	28/min	22/min	20/min	18/min
SPO2	100%	100%	100%	98%	95%	95%
TEMP	100.2F	101.5F	100F	99F	98.6F	98.6F

ECG on 21/3/25

Chest X-ray on 21/3/25

CT Finding on 23/3/25

Echocardiography and progression of patient’s cardiac status

Discussion

PPCM is taken into consideration as a diagnosis of exclusion in women who present with HF because of left ventricular (LV) systolic dysfunction when no apparent reason is detected. Four factors are included in the definition of PPCM: 1) onset of heart failure during the final month of pregnancy or within five months after delivery; 2) lack of a known cause for the heart failure; 3) lack of discernible heart disease prior to the final month of pregnancy; and 4) left ventricular (LV) dysfunction (ejection fraction <45% or reduced shortening fraction). Multiple gestation, multiparity, advanced age, gestational hypertension, preeclampsia, or eclampsia are few suggested risk factors.

Multifactorial pathophysiology is present. Several potential causes, such as myocarditis, autoimmune, vascular dysfunction, and a hereditary propensity shared with dilated cardiomyopathy, the exact pathophysiologic mechanism of PPCM is yet unknown. More recently, it has been hypothesized that a significant mechanism causing endothelial damage that initiates and drives PPCM may be the oxidative stress-mediated cleavage of the nursing hormone prolactin into a smaller physiologically active sub fragment. It has been suggested that PPCM could be an inflammatory reaction brought on by pregnancy. In certain instances, increased levels of interleukin-6 and tumor necrosis factor-alpha support this idea. Genetic factors play a significant role too.

Clinical characteristics of PPCM include chest pain and signs of congestive heart failure. Tachycardia, tachypnea, pulmonary rales, an enlarged heart, and an S3 heart sounds are some of detectable symptoms. Some severe cases may have significant arrhythmias, while others may present in cardiogenic shock, which is similar to disorders like myocardial infarction (MI), pulmonary embolism (PE), or amniotic fluid embolism.

Since the LVEF is usually less than 45%, Echocardiography should be done in each suspected case. The ECHO may show pulmonary hypertension, left atrial or Bi-Atrial enlargement, functional mitral and/or tricuspid regurgitation, LV and right ventricular dilatation and/or dysfunction, and systolic dysfunction. PPCM is typically associated with dramatically raised levels of brain natriuretic peptide (BNP) and N-terminal pro-BNP, which do not alter appreciably during normal pregnancy. A normal ECG does not rule out PPCM. Pulmonary venous congestion is seen on chest X-rays. Cardiac MRI can provide precise measurements of the chamber and ejection fraction.

Pre-existing cardiac abnormalities such as cardiomyopathy, valvular (mostly stenosis) or congenital heart disease, acute pulmonary embolism, and acute coronary syndromes are excluded as differential diagnosis making PPCM as Diagnosis of Exclusion.

The “BOARD” acronym, which stands for bromocriptine, oral heart failure medications, anticoagulation, relaxants, and diuretics, refers to treatment options based on the severity of heart failure and emphasizes on the health of both mother and fetus. The results of PPCM have been shown to improve with the administration of bromocriptine, a medication that selectively inhibits the production of prolactin. Dietary salt restriction is the first line of treatment for fluid management, although loop diuretics are safe for people with lung or peripheral edema.

As beta 1-selective medications, such as metoprolol, interfere less with beta 2-mediated peripheral vasodilation and uterine relaxation, they are recommended. Beta-blockers, hydralazine, and digoxin are safe oral heart failure medications. Patients with atrial fibrillation, left ventricular thrombus, systemic embolism, bromocriptine use, and an LVEF < 30% may be candidates for anticoagulant therapy.

For the treatment of severe cases of cardiovascular compromise, patients who exhibit hemodynamic instability despite inotropic support should be evaluated for temporary mechanical circulatory support, such as extracorporeal membrane oxygenation (ECMO) or ventricular assist device therapy.

Conclusion

PPCM is a rare but a life-threatening condition which should be investigated in women developing symptoms of heart failure with systolic dysfunction during pregnancy. Health Care Professionals should have heightened vigilance for the atypical presentation and prompt treatment with maternal and fetal life outcome should be established. This case highlights on significance of initiation of early conservative strategies including aggressive fluid resuscitation with administration of broad-spectrum antibiotics and mitigating the cardiac function as most patients recover the systolic function with time.

Close collaboration with the Obstetric team, Cardiology team and on foot efforts of the ICU team could bring us to the desired result. Keeping ourselves educated and improving skills towards idiopathic conditions like PPCM which go unnoticed is mandatory for future outcomes.

Bibliography

Challenges in the diagnosis of peripartum cardiomyopathy: a case series Fabio Chirillo, Anna Baritussio , Umberto Cucchini , Ermanno Toniolli , Angela Polo , Antonio Iavernaro , European Heart Journal – Case Reports, Volume 5, Issue 2, February 2021, ytab001, https://doi.org/10.1093/ehjcr/ytab001
Peripartum Cardiomyopathy: A Review of Three Case Reports Ankita Kumari, Saroj Singh, Shikha Singh, Mridul Chaturvedi.
Peripartum Cardiomyopathy: Case Report Case Report Asli Mohamed Abdullahi* , Fatima Ibrahim Nor, Ahmed Mohamed Abdulle and Abdirahman Hussein Ali Dr. Sumait Hospital, SIMAD University, Somalia
Davis, M, Arany, Z, McNamara, D. et al. Peripartum Cardiomyopathy: JACCState-of-the-Art Review. 2020 Jan, 75 (2) 207–221.https://doi.org/10.1016/j.jacc.2019.11.014
Perea rojas, d., seni hernandez, c., rojas torres, i., olivares olmos, m., garcia jarava, c., gaivao arciniegas, d., seni hernandez, s., corrales calderon, l., perea vasquez, l., salva camano, S.. Peripartum Cardiomyopathy: A Case Report of Mortality From a Rare and Potentially Fatal Condition. Journal of Medical Cases, North America, 15, jul. 2024. Available at: <https://www.journalmc.org/index.php/JMC/article/view/4228/3613>. Date accessed: 18 Apr. 2025.

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An intriguing challenge of unveiling the unexpected: Peripartum cardiomyopathy