Giant-Size Main Pulmonary Artery Aneurysm in an Adult Patient with Ebstein Anomaly and Dextrocardia

Main pulmonary artery aneurysms are rare,mostly asymptomatic and discovered accidentally.The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension,Behcet’s disease,connective tissue disorders,congenital heart disease,vasculitis,syphilis,tuberculosis and endocarditis.There are some indices that dextrocardia is associated with anomalies of the pulmonary arteries and pulmonary valve.A rare occurrence of main pulmonary artery aneurysms results in a lack of recommendations,so the remaining challenges are whether main pulmonary artery aneurysms should be treated,how,and when.The decision on surgical intervention or conservative treatment of the main pulmonary artery aneurysms depends on its size,etiology and accompanying diseases and includes a multidisciplinary heart team.Our case of the main pulmonary artery aneurysm and pulmonary valve abnormality associated with Ebstein anomaly and dextrocardia in a 67-year-old male patient brings causality considerations and treatment options in such a unique condition.

Six-Year Outcome after Valve Replacement and Resynchronization Therapy in TGA Patient

Patients with complete transposition of the great arteries(TGA)treated by the Senning procedure have a higher risk of developing heart failure due to:a)additional work load of the systemic(morphologic right)ventricle(sRV),b)arrhythmias,mainly caused by surgical implications at the atria as well as c)worsening of systemic tricuspid regurgitation.We present a unique case of a female patient who developed all these complications,who was successfully treated and was able to carry out a twin pregnancy.This breakthrough approach was based on:1.detecting reversibility potential of myocardial systolic dysfunction in a severe valvular lesion combined with continuous systemic afterload settings and permanent tachyarrhythmia,and 2.prevention of subsequently iatrogenic worsening of systemic ventricular function due to permanent pacing.Surgical replacement of systemic tricuspid valve(sTV)and cardiac resynchronization device(CRT)implantation after nodal ablation resulted in recovering of the systolic function and a positive remodeling of the sRV.The reversal of a further decline in systolic function was achieved by permanent arrhythmia control,synchronous pacing with epicardial leads of CRT,sTV replacement as well as echocardiographic monitoring during pregnancy to determine the right time for delivery.Two years after delivery,the patient remains in NYHA Class I.