Post-Partum Dyspnea in a Previously Asymptomatic 22 Year Old: Management of Rapidly Progressive Pulmonary Arterial Hypertension in the Acute and Post Acute Setting

Special Article - Pulmonary Hypertension

Austin J Clin Med. 2016; 3(1): 1028.

Post-Partum Dyspnea in a Previously Asymptomatic 22 Year Old: Management of Rapidly Progressive Pulmonary Arterial Hypertension in the Acute and Post Acute Setting

Johnson AE¹, Rivera-Lebron BN², Risbano MG², Holtz JE², Teuteberg JJ², Ramani RN¹, Songdechakraiwut T³, Shah DK³, Hickey GW¹, Lagazzi LF³, Berlacher KL¹ and Simon MA¹*

¹Heart and Vascular Institute, University of Pittsburgh Medical Center, USA

²Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

³Department of Cardiothoracic Surgery, Division of Cardiac Surgery, UPMC Presbyterian, USA

*Corresponding author: Simon MA, Heart and Vascular Institute, University of Pittsburgh Medical Center, 200 Lothrop Street, Scaife Hall, Pittsburgh, USA

Received: September 21, 2016; Accepted: October 07, 2016; Published: October 10, 2016


A 22-year-old woman was transferred from another hospital after developing precipitous postpartum hypoxemia and hypotension. Upon arrival, the patient was severely hypoxic with oxygen saturation under 20% despite mechanical ventilation. Physical examination was notable for signs of right heart failure. Progressive bradycardia and asystolic arrest ensued. Prolonged resuscitation, necessitated cannulation for veno-venous Extra Corporeal Membrane Oxygenation (ECMO). A patent foramen ovale with right to left shunt and elevated pulmonary pressures was identified by transesophageal echocardiogram. Right to left intracardiac shunting can be seen in chronic Pulmonary Arterial Hypertension (PAH), but does not fully explain the severe acute decompensation. Amniotic Fluid Embolism (AFE) causes acute decompensation after delivery necessitating advanced resuscitative efforts. ECMO can serve as supportive therapy in unstable patients with AFE and PAH. This case highlights supporting evidence for AFE in the unique setting of severe PAH, as well as its diagnosis and management. Medical therapy for PAH is also reviewed.

Keywords: Pulmonary arterial hypertension; Amniotic fluid embolism; Patent foramen ovale; Intracardiac shunt


AFE: Amniotic Fluid Embolism; PAH: Pulmonary Arterial Hypertension; TTE: Transthoracic Echocardiogram; ECMO: Extracorporeal Membrane Oxygenation; PE: Pulmonary Embolism; PA: Pulmonary Artery; PAH: Pulmonary Arterial Hypertension; RV: Right Ventricle; RVH: Right Ventricular Hypertrophy; LV: Left Ventricular; PFO: Patent Foramen Ovale; PCWP: Pulmonary Capillary Wedge Pressure

Case Presentation

History of present illness

A 22-year-old woman was transferred to our quaternary care facility with severe hypoxemia after childbirth. She had been reportedly healthy prior to and during pregnancy but noted mild dyspnea when climbing stairs toward the end of the pregnancy. Approximately 7 hours after a routine, full term, spontaneous vaginal delivery, the patient developed acute onset of respiratory failure with refractory hypoxemia necessitating intubation. She was initiated on vasopressors for shock. A CT angiogram of the chest ruled out Pulmonary Embolism (PE), but revealed Pulmonary Artery (PA) enlargement, concerning for Pulmonary Arterial Hypertension (PAH). Due to persistent hypoxia, the patient was started on NO and transferred to our facility.

Additional medical history

The patient was gravida 1, para 1. She had a history of childhood asthma, endometriosis status post laparoscopic removal, and migraine headaches. Prior to hospitalization, she was taking prenatal vitamins and no other medications. Family history was notable for 2 uncles who died from unknown congenital heart disease. The patient smoked a half pack of cigarettes per day since age 15, but quit during pregnancy.

Physical examination

Vital signs on admission were: temperature 37 degrees, heart rate 141 beats per minute, blood pressure 79/46 mmHg, oxygen saturation 60%. Ventilator settings were rate of 22 breaths per minute, 100% FiO2, and PEEP of 15 cm of water. The patient was intubated and comatose. Pupillary response was normal. The neck was supple and the jugular venous pressure did not appear elevated. She was breathing at the rate of the ventilator. There were no adventitial lung sounds. There was a fixed split second heart sound, but no murmurs, rubs, or gallops. There was no sternal lift and the point of maximal impact was not displaced. The digits were mildly cyanotic, but there was no clubbing or edema.

Initial acute management

Severe hypoxia persisted and oxygen saturation deteriorated despite suctioning, ventilator recruitment maneuvers and bag-valve. A bronchoscopy excluded the presence of obstructive mucous plugs. The heart rate became progressively bradycardic. She then developed pulseless electrical activity and asystole. Resuscitative measures included chest compressions and epinephrine bolus then continuous infusion. The patient regained spontaneous circulation briefly then again became bradycardic and pulseless.

Upon resumption of chest compressions, the patient’s oxygen saturation would improve to the 40-50% range. However spontaneous circulation and oxygen saturations were short-lived as the patient repeatedly required CPR. The patient was placed on vasopressor support with epinephrine, norepinephrine, and vasopressin infusions.

A bedside Transthoracic Echocardiogram (TTE) revealed massively dilated Right Ventricle (RV) with severe diffuse hypokinesis and Right Ventricular Hypertrophy (RVH). The right atrium was dilated. There was malcoaptation of the visualized tricuspid leaflets. Left ventricular (LV) systolic function was mildly decreased.

Due to severe hypoxia, veno-venous ECMO was initiated with flows of 5L. Oxygen saturation improved to 90%.

Laboratory data and studies

Arterial blood gas on arrival: pH 7.21, PaCO2 49mmHg, PaO2 <30mmHg, HCO3 19mEq/L, base deficit 8mEq/L, SO2 18%. Troponin was only mildly elevated at 0.68. A basic metabolic panel was normal. Lactate was 3.7 and increased to 12.3 minutes later. Complete blood count was significant for leukocytosis of 17, 900cells/uL, hemoglobin 9.2g/dL and platelets 82,000/uL. INR was 2.0. Bronchoalveolar lavage 4 days after admission showed many white blood cells but normal respiratory flora.

Electrocardiogram upon arrival revealed sinus tachycardia, right atrial abnormality, right axis deviation, and incomplete right bundle branch block. Chest x-ray showed diffuse hazy lung opacities. Transesophageal echocardiogram performed the day after admission revealed normal LV size and thickness. LV systolic function was mildly hypokinetic with an ejection fraction of 45%. There was flattening of the intraventricular septum consistent with RV pressure overload (Figure 1). RV function was severely decreased with RVH. The right atrium was giant. There was a persistent Eustachian valve (Figure 2) and a patent foramen ovale (0.7cm diameter) with a right to left shunt (Figure 3). The main PA was severely dilated at 3.8cm with echodensity suspicious for embolic material. PA catheter confirmed a pressure of 97/59mmHg.