What is the management approach for a patient diagnosed with cardiomyopathy peripartum?

Management of Peripartum Cardiomyopathy

Treat peripartum cardiomyopathy aggressively with standard heart failure therapy, prioritizing rapid stabilization with oxygen, diuretics, and vasodilators for acute presentations, while avoiding ACE inhibitors during pregnancy but transitioning to them immediately postpartum. 1

Acute Stabilization

Immediate Interventions for Symptomatic Patients

• Administer oxygen therapy to maintain arterial oxygen saturation ≥95%, using non-invasive ventilation with PEEP 5-7.5 cmH2O if hypoxemia or pulmonary edema is present 1
• Give intravenous furosemide 20-40 mg IV bolus for congestion and volume overload 1
• Use intravenous nitroglycerin 10-20 up to 200 μg/min if systolic blood pressure is >110 mmHg; exercise caution if SBP is 90-110 mmHg 1
• Consider inotropic agents (dobutamine or levosimendan) in patients with signs of hypoperfusion or persistent congestion despite vasodilators and diuretics 1

Advanced Support for Refractory Cases

• Consider mechanical circulatory support if the patient remains dependent on inotropes or intra-aortic balloon pump despite optimal medical therapy 1
• LVAD may be used as a bridge to recovery or transplantation, particularly since PPCM has a higher recovery rate than other forms of dilated cardiomyopathy 1
• Cardiac transplantation should be considered if weaning from mechanical support is unsuccessful 1

Medical Management Strategy

During Pregnancy (Antepartum)

• Use hydralazine and long-acting nitrates for afterload reduction since ACE inhibitors and ARBs are contraindicated due to fetal toxicity 1
• Beta-blockers (preferably β1-selective) can be used safely during pregnancy 1
• Consider anticoagulation due to the pro-thrombotic nature of PPCM, especially with severely reduced ejection fraction 1

After Delivery (Postpartum)

• Transition immediately to standard heart failure medications per current guidelines, including ACE inhibitors or ARBs, beta-blockers, and aldosterone antagonists 1
• Bromocriptine may be considered postpartum to enhance cardiac function recovery, but must be accompanied by prophylactic anticoagulation 1
• If breastfeeding is chosen, use captopril, enalapril, or quinapril as these ACE inhibitors have been adequately tested in breastfeeding women 2, 1

Critical Medication Considerations

Breastfeeding is not advised based on postulated negative effects of prolactin subfragments, though this practice is not fully evidence-based 2

Labor and Delivery Management

Timing and Mode of Delivery

• Allow spontaneous vaginal birth for stable patients with well-controlled cardiac condition and apparently healthy fetus 2, 1
• Perform planned cesarean section for critically ill women requiring inotropic therapy or mechanical support 2, 1
• Consider urgent delivery regardless of gestation in women with advanced heart failure and hemodynamic instability, prioritizing maternal cardiovascular benefit 2

Intrapartum Monitoring and Support

• Conduct labor in a high-care area with experience managing cardiac disease in pregnancy 2, 1
• Use continuous invasive hemodynamic monitoring with continuous urinary catheter drainage 2, 1
• Prevent fluid overload and pulmonary edema from IV infusions through careful fluid management 2
• Continue antenatal oral medications but stop heparin after contractions start 2

Analgesia and Anesthesia

• Use epidural analgesia during labor as it stabilizes cardiac output 2, 1
• For cesarean section, use continuous spinal anesthesia or combined spinal-epidural anesthesia 2

Labor Stage Management

• Position patient in left lateral position to ensure adequate venous return from the inferior vena cava, though sitting-up position may be needed for women in cardiac failure 2
• Avoid prolonged bearing down efforts during the second stage 2, 1
• Use low forceps or vacuum-assisted delivery to shorten the second stage if spontaneous delivery cannot be achieved rapidly 2, 1
• Manage third stage actively with single dose of intramuscular oxytocin; ergometrine is contraindicated 2, 1

Immediate Postpartum Management

• Give a single IV dose of furosemide after delivery to manage auto-transfusion of blood from lower limbs and contracted uterus 2, 1
• Restart anticoagulants in consultation with obstetrician and anesthesiologist when postpartum bleeding has stopped and epidural/spinal catheter has been removed 2

Diagnostic Workup

Essential Imaging

• Perform echocardiography immediately to establish diagnosis and obtain prognostic information 2
• LV end-diastolic diameter >60 mm predicts poor recovery of LV function, as does LVEF <30% 2
• Repeat echocardiography before discharge and at 6 weeks, 6 months, and annually to evaluate treatment efficacy 2

Additional Testing

• Obtain ECG as it is seldom normal on presentation in heart failure; 96% show ST-T wave abnormalities 2
• Measure BNP or NT-proBNP as all PPCM patients have elevated levels compared to healthy postpartum mothers 2

Prognosis and Follow-up

Expected Outcomes

• Mortality rates vary geographically: 6-month mortality ranges from 10% in South Africa to 14-16% in Brazil and Haiti, with 2-year mortality reaching 28% in some populations 2
• LV systolic function returns to normal in 23-54% of patients across different case series 2
• Most pregnancy-related deaths occur in the first 4 weeks postpartum, requiring close monitoring during this period 1

Subsequent Pregnancy Counseling

• Advise against subsequent pregnancy if LV function has not normalized, as development of heart failure symptoms occurs in 44% versus 21% in those with normalized LVEF 2
• Three deaths occurred in women with persistently low LVEF entering subsequent pregnancy, whereas none died with normalized LVEF 2
• Provide careful family planning counseling as women with PPCM are usually in the middle of family building 2

Common Pitfalls to Avoid

• Do not delay diagnosis by attributing dyspnea and edema to normal pregnancy symptoms 2
• Never use ergometrine for third stage management as it is contraindicated 2, 1
• Do not continue ACE inhibitors or ARBs during pregnancy due to fetal toxicity 1
• Avoid excessive IV fluids during labor to prevent pulmonary edema 2