INTRODUCTION: Atrial Septal Defect (ASD) is the commonest adult congenital heart defect (CHD). 15 % of these patients will eventually develop pulmonary hypertension (PHTN) if left untreated. ASD closure is not recommended when PHTN is irreversible. We present a patient with ASD and irreversible PHTN who was treated with Epoprostenol. She underwent percutaneous closure of her ASD with an altered closure device to allow right to left shunting with exercise.
CASE PRESENTATION: 35 yr female presented with 1 year of worsening dyspnea, presyncope with activity and inability to climb one flight of stairs. Her past medical history is notable for 2 pregnancies. During her second she experienced a pulmonary embolism and was treated with anticoagulation for 6 months. On examination BP 102/70, HR 90/min and O2 Sat 99%. Lung fields were clear and cardiac exam revealed prominent second heart sound with fixed splitting of both components. She had mild clubbing, no edema. A TTE performed showed a dilated RV and ostium secundum ASD. Her workup for other causes of pulmonary hypertension was negative. Cardiac catheterization demonstrated severe PHTN. She was started on IV Epoprostenol and was titrated to 33ng/kg/min. At 6 months her 6 minute walk, functional class and hemodynamics improved. 2 yrs later she remained on Epoprostenol at 42 ng/kg/min. Her functional status and hemodynamics continued to improve with titration. Due to the desaturation with exercise it was felt that she had a right to left shunt with exercise. As a result she underwent partial closure with a fenestrated 34 mm Amplatzer Septal Occluder. A covered stent was placed through the ASD device and ballooned to 9mm to maintain a pop-off for potential right to left shunting. She is currently on Epoprostenol with NYHA II functional status.
DISCUSSIONS: Atrial Septal Defects account for 18.8 % of CHD in the United States. It is more common in women. Most patients reach adulthood; however if it is not surgically corrected the mean age of death is 37.5 ± 4.5 years. Dyspnea is the most common presenting symptom. Alternative presentations include CNS manifestations from paradoxical emboli, supraventricular arrhythmias or recurrent pulmonary infections. Evaluation is performed utilizing TTE with contrast, a TEE or cardiac MRI to better estimate the location, defect size and shunt. Cardiac catheterization is performed to establish hemodynamic parameters and evaluate for reversibility. ASD closure is selected in patients whose Qp/Qs ratio is > 1.5. Patients with irreversible PHTN have higher mortality with closure compared to medically managed patients. Irreversible PHTN is suggested by a Qp/Qs ratio of less than 1, PVR > 15 and/or a Rp:Rs <0.33. Desaturation with exercise is suggestive of a right to left shunt, one that occurs from necessity due to high PVR impairing right sided cardiac output. The use of selective pulmonary vasodilators improve survival in patients with PAH and have shown to be beneficial in patients with shunt related PHTN. These agents are vasodilators and may over time remodel the pulmonary vasculature. In this case we were able to demonstrate improved hemodynamics with Epoprostenol over time followed by a partial closure of the ASD. The partial closure was facilitated by the addition of the stent within the device itself, effectively allowing a right to left shunt when needed.
CONCLUSION: Congenital Heart Disease should be considered in the evaluation of dyspnea in a young adult. The management of ASD with associated PHTN is difficult. It is pertinent that a detailed hemodynamic assessment be undertaken. The current vasodilator therapies may have role in making inoperable candidates operable. Manipulation of the device allowing right to left shunting contributed to the optimal outcome in this case.
DISCLOSURE: Jarrod Bruce, No Financial Disclosure Information; No Product/Research Disclosure Information