An 18-year-old female patient with a past medical history significant for iron deficiency anemia presented four-week postpartum (first pregnancy) with a one-week history of chest pain and shortness of breath. The patient described a left-sided chest pain as not radiating, constant and stabbing in nature, moderate to severe, pleuritic, and not related to physical activity. The pain was associated with worsening shortness of breath and palpitations. The patient reported associated exertional dyspnea, dizziness, and fatigue that restricted her ability to take care of her baby. She denied fever, orthopnoea, paroxysmal nocturnal dyspnea, or lower limb swelling. The patient had recently returned from a twenty-eight-hour car journey. Apart from anemia, her pregnancy and delivery were both uneventful. The patient has no allergies, no family history of cardiovascular, or clotting disorders.
On examination, the patient was distressed, tachycardic, and tachypneic and had normal blood pressure. She required FiO2 of 44% to maintain normal saturation. Heartbeat was regular: soft heart sounds without murmurs, gallops, or rubs. There was no parasternal heave and no clinical signs of fluid overload. Moreover, there were no clinical features of endocarditis. She had normal vesicular breath sounds bilateral, with no crackles or wheezes. Lower limb examination was normal without signs of deep vein thrombosis.
Her EKG showed sinus tachycardia with T wave inversion in leads V1-3 (Figure 1).
Biochemical investigations showed a D-dimer of 3331 ng/ml (<500 ng/ml), BNP of 1034 pg/ml (<125 pg/ml), mildly elevated troponin I, and microcytic anemia. Otherwise, renal function, electrolytes, liver function tests, and thyroid hormones were within normal limits. Transthoracic echocardiogram showed a severely dilated right ventricle with globally reduced right ventricular systolic function and a left ventricular ejection fraction of 45-50% (Figure 2). CT pulmonary angiogram showed a dilated pulmonary trunk with no evidence of pulmonary embolism (Figure 3). An extensive venous thromboembolic workup including ultrasound Doppler of both lower limbs, CT venogram of the pelvis and lower limbs, and V/Q scan was negative.
Cardiac MRI showed a severely dilated right ventricle with a strain pattern with significant global reduced right ventricular systolic function, no signs of dysplasia or myocarditis, normal coronary anatomy, and no septal defects. Coronary angiography showed no coronary artery disease or dissection. Right heart catheterization (RHC) revealed the following: right atrial pressure = 12 mmHg (normal range 1-8 mmHg), right systolic ventricular pressure = 45 mmHg (normal range 15-30 mmHg), mean pulmonary artery pressure = 31 mmHg (normal range 10-22), pulmonary capillary wedge pressure = 7 mmHg (normal range 6-15), cardiac output = 2.97 L/min (normal range00204-8 mmHg), and cardiac index of 1.74/min/m2 (normal range 2.8-4.2), respectively.
Eventually, the patient was diagnosed with postpartum pulmonary hypertension. She was admitted and treated in the intensive care unit under the direct care of a pulmonary hypertension team. Her management was guided by a close follow-up of her hemodynamics. Given the high risk of sudden deterioration at any given point, inotropic support was considered and planned. Luckily, the patient's blood pressure remained normal. Good perfusion pressure was supported by normal lactate measures and absence of end-organ damage. Intravenous furosemide infusion was initiated with close monitoring of volume status, which was well tolerated without the need for inotropic support along with parenteral iron. The patient was discharged on torsemide and spironolactone.
On review three weeks postdischarge, her functional status was back to premorbid levels with improved exercise tolerance. Repeated transthoracic echocardiogram revealed no significant interval changes. Repeat right heart catheterization showed mild pulmonary hypertension, normal right and left ventricular filling pressures, and normalization of the cardiac output and index. The patient was counseled about the importance of contraception and the risks of pregnancy, given the associated mortality risk. However, a few months later, the patient became pregnant, and after a multidisciplinary meeting and recounseling, she underwent a therapeutic abortion. She remains to follow in the pulmonology clinic and is doing well.