A Fatal Traid: Eisenmenger's Syndrome, Pregnancy, and Severe Preeclampsia

INTRODUCTION Eisenmenger’s syndrome (ES), named after Victor Eisenmenger who first identified the syndrome in 1897, was described in 1958 by Dr. Wood as elevated pulmonary arterial pressure and pulmonary vascular resistance,... [ view full abstract ]

INTRODUCTION
Eisenmenger’s syndrome (ES), named after Victor Eisenmenger who first identified the syndrome in 1897, was described in 1958 by Dr. Wood as elevated pulmonary arterial pressure and pulmonary vascular resistance, resulting in a reversed or bidirectional shunt at any site between the two circulation systems. The shunting is most commonly a result of congenital heart defects (CHD). Ventricular Septal Defect (VSD) is the most common CHD and also the most lethal ES CHD with a mortality rate as high as 66%.

CASE
A 23-year-old primigravida with no prenatal care presented to the emergency room with worsening shortness of breath over the last three days. Her initial vitals were: Temp 98.2 oF, HR 122 bpm, BP 174/89 mmHg, RR 48 rpm, and O2Sat 60%. The patient quickly became unstable and with O2 saturation worsening, the patient was intubated and placed on BiPAP ventilation. Labs drawn showed Hgb/Hct 14.7/44, Plt 17,000, normal AST/ALT, Fibrinogen 536, and normal PT/PTT/INR.
Evaluation of the fetus revealed a category III fetal heart tracing with a fetal heart rate of 150bpm, absent variability, and recurrent variable decelerations. Fundal height was consistent with a 30-34 week fetus. Due to worsening blood pressure, heart rate, and oxygen saturation, a stat primary low transverse cesarean section was performed and resulted in a liveborn male, APGAR 2/7, weight 2120g, and Ballard scores corresponded to a 35-week gestation. Estimated blood loss was 800mL.
Postoperatively the patient was stable with O2 sat 80-90% but remained intubated and was sent to surgical ICU. Magnesium sulfate was started for seizure prophylaxis as the patient had preeclampsia with severe features. Lower extremity venous dopplers were negative. A chest XRAY showed left upper lobe infiltrates and edema, suggestive of pneumonia and she was started on broad-spectrum antibiotics. Patient experienced intermittent periods of hypoxia despite BiPAP that resolved with positioning which prompted an echocardiogram and bubble study that showed a LVEF of 40 to 45% and a right-to-left shunt, suggestive of a large secundum ASD.
On post op day 1, difficulties ventilating the patient persisted and repeat chest XRAY showed worsening bilateral infiltrates and pulmonary vascular congestion despite antibiotic treatment for pneumonia. Thrombocytopenia persisted and the she received a total of 4 units of platelets. A transesophageal echocardiogram showed a LVEF of 70 to 75%, 5mm muscular VSD, 5mm patent foramen ovale, and loculated pericardial effusion near the left ventricular apex. Patient was diagnosed with Eisenmenger syndrome with acute respiratory failure.
On post op day 3, after inability to adequately ventilate and multiple codes, the family asked to withdraw support and the patient died from acute hypoxic respiratory failure

DISCUSSION
With improved access to pediatric cardiology and cardiac surgery, congenital heart defects are diagnosed earlier and better managed at a younger age. Improved outcomes translate to an increasing number of individuals reaching their reproductive years. In the CONCOR registry out of the Netherlands they estimated the prevalence of pulmonary arterial hypertension to be 4.2% and only 1% for Eisenmenger’s Syndrome.
Pregnancy is contraindicated in women with CHD as the risk of developing pulmonary artery hypertension and a right to left shunt increase due to the hemodynamic changes associated with pregnancy. Mortality is estimated to be as high as 60%. SVD is the most common CHD with atrial septal defect and patent foramen ovale less commonly. Preeclampsia is a rare confounding variable with only five cases reported in current literature. Of those five, only one survived but the difference being the CHD was a patent ductus arteriosus. There are no cases reported in current literature of maternal survival with Eisenmenger’s syndrome, severe preeclampsia, and a VSD.
Current recommendation regarding CHD and fertility includes long-acting reversible contraception or permanent sterilization. If pregnancy does occur, close management between cardiology and OB/GYN must occur and recommendation for inpatient management beginning at 20 weeks gestation.
This case is unique in that it represents the frightening possibility of CHD that was never diagnosed in a now pregnant female. With no prior medical history and no prenatal care we were unable to offer medical abortion at an earlier gestation age, let alone counsel against pregnancy.