Background
Eisenmenger syndrome is a clinical condition, in which congenital heart defect (CHD) with persistent intracardiac shunt results in development of pulmonary hypertension. With time right-to-left shunt becomes reversed leading to development of cyanosis. Eisenmenger s. may be caused by a number of different CHDs including various forms of ventricular or atrial septal defects, patent ductus arteriosus, aortopulmonary window etc. or as a result of surgical correction of CHDs with formation of systemic-to-pulmonary shunts. The overall prognosis for Eisenmenger patients is serious leading to marked impairment of physical activity, their life expectancy is reaches 20-50 years.
Transposition of the great arteries (TGA) is a ventriculoarterial development disorder, in which the aorta arises from the right ventricle and the pulmonary artery from the left. Two parallel circulation exists where deoxygenated systemic venous blood flows through the right atrium and back to the systemic circulation via the right ventricle and aorta, and second with oxygenated pulmonary venous blood via the left atrium and back to the lungs via the left ventricle and pulmonary artery. Therefore, peripheral cyanosis is the most prominent sign, however its severity depends on the amount of intercirculatory mixing of the blood.
Frequently, TGA is accompanied with other cardiac anomalies or functional defects. Ventricular septal defect (VSD) occurs in about 50 percent of TGA cases.
Management of TGA is surgical. Most patients are referred for surgical repair during the first three to five days of life. The choice of surgical procedure is dependent on the presence and nature of other cardiac anomalies.
Potential follow-up complications include those related to the procedure like main and branch pulmonary artery stenosis, neo-aortic root dilation and regurgitation, coronary artery stenosis or insufficiency etc. or those associated with progression of the disease or development and progression of Eisenmenger s including dyspnea, chest pain, palpitations or syncope.
Case presentation
We present a 23 year-old patient with Eisenmenger’s symdrome. The patient was born at term. Cyanosis was present right after the delivery. Subsequent diagnosis of complex cyanotic congenital heart defect: transposition of great arteries with hemodynamic single ventricule was made. Pulmonary banding was performed at the age of one but turned out to be unsuccessful what eventually led to pulmonary hypertension.
Right heart catheterization was performed at the age of 13 and confirmed pulmonary hypertension with mean pulmonary arterial pressure (mPAP) of 103mmHg. Sildenafil therapy was initiated and after 5 years bosentan was added to treatment due to lack of satisfactory improvement.
Currently the patient is in WHO functional class II. Periodically complains of headaches, dizziness (hyperviscosity syndrome), what requires phlebotomy once a year. He reaches distance of 480m in 6 minutes walking test. His hemoglobin level is maintained between 20-23g% and hematocrite between 65-72%. Echocardiographic evaluation discloses hemodynamically common one- chamber vantricle with preserved residual part of the septum from the apex, enlarged both right and left atrium, d-transposition of the great arteries and pulmonary artery aneurysm of 71mm of diameter with concomitant pulmonary valve regurgitation.
The presence of an aneurysm was first confirmed in computed tomography (CT) and magnetic resonance (MRI) of the chest in the age of 17.
Since then, the size of the aneurysm is maintained at a steady level.
For the last year he has begun to complain of a chest pain not related to exercise.
In January 2013 he underwent cardiopulmonary testing, which revealed low exercise tolerance with maximal oxygen uptake at peak exercise ( O 2peak ) of 14.7ml/kg/min, which was lower compering to the previously performed in 2012 (17ml/kg/min).
We referred to the panel of experts with our concern whether this patient should be qualified for an early heart and lung transplant due to pulmonary artery aneurysm?
Current guidelines
No clear guidelines for management of PA aneurysm in patients with Eisenmeger syndrome and TGA are now available.
Expert’s comments:(Written authorization required from each expert)
1. Prof. A. Biederman
It is recommended to do cardiosurgery consult – qualification for heart-lung transplantation. Danger of pulmonary artery aneurysm rupture is high and therefore may be an additional indicator for consideration of early transplant.
2. G. Kopeć
Presence of this big pulmonary artery aneurysm is a real threat of coronary arteries compression. It is recommended to perform coronary CT angiography to evaluate coronary circulation. V/Q scintigraphy might be also useful to show any signs of ischemia.
Expert’s conclusions:
Patient will be consulted towards heart – lungs transplantation qualification. Coronary CT angiography will be performed to evaluate coronary circulation to decide whether any stenting procedure is required.
Authors:
Mroczek Ewa1, Furdal Michał1, Jakub Stępniewski2
Experts:
Prof. A. Biederman , G. Kopeć
1Cardiology department Regional Hospital in Wroclaw, Poland
2Department of Cardiac and Vascular Diseases, John Paul II Hospital in Krakow, Poland
