Congenital defects/diseases Background: Coronary-to-pulmonary artery fistulas are congenital coronary anomalies that are often incidentally found and no hemodynamic significance. They are abnormal vessels originating from the coronary artery and terminating into the pulmonary artery, with a tortuous pathway. However, coronary-pulmonary artery fistula associated with coronary artery aneurysm is a rare condition that can cause cardiac death. Case Report: A 55-year-old Thai woman diagnosed with traumatic aortic injury after being gored by a buffalo was incidentally found by computed tomography (CT) to have a coronary arteriovenous fistula with large aneurysm. One week after urgent thoracic endovascular aortic repair (TEVAR), she had a cardiac tamponade. Median sternotomy was performed for diagnosis and treatment. The final diagnosis was rupture of a large aneurysm of the coronary-pulmonary artery fistula (CPAF). No complications were found and the patient was discharged 2 weeks after surgery. Conclusions: CPAF associated with rupture of a giant coronary aneurysm is a rare and life-threatening condition. Prompt diagnosis and emergency surgery are essential to treatment of these patients.
Patient: Male, 34-year-old
Final Diagnosis: Pulmonary stenosis and heart failure 24 years after surgical correction with the Rastelli procedure for congenital D-TGA, VSD, and pulmonary atresia
Symptoms: Dyspnea on exertion
Medication:—
Clinical Procedure: Pulmonary artery plasty • Rastelli procedure • Right-ventricle-to-pulmonary-artery conduit • VSD closure
Specialty: Anatomy • Cardiac Surgery • Cardiology • General and Internal Medicine
Objective:
Congenital defects/diseases
Background:
Dextro-transposition of the great arteries (D-TGA) with a ventricular septal defect (VSD) and pulmonary atresia is an uncommon congenital conotruncal abnormality. Surgical correction is performed using the Rastelli procedure, which includes a ventricular septal patch to direct blood from the left ventricle to the aorta and a valved conduit to connect the right ventricle to the pulmonary artery. This report is of a 34-year-old Thai man who presented with pulmonary stenosis and heart failure 24 years after surgical correction with the Rastelli procedure for congenital D-TGA, VSD, and pulmonary atresia.
Case Report:
A 34-year-old Thai man presented with dyspnea on moderate exertion. His cardiovascular examination revealed a median sternal surgical scar, parasternal heaving, a grade III systolic ejection murmur at the left upper parasternal border, and a single second heart sound. Echocardiography demonstrated degenerative calcification of a severely stenosed pulmonary valve and impaired right ventricular function. A color Doppler M-mode echocardiogram showed VSD patch leakage. A computed tomography scan with 3-dimensional heart reconstruction demonstrated a significantly stenosed branch pulmonary artery. Right and left heart catheterization confirmed the multi-site stenoses were hemodynamically significant. The patient underwent surgery for VSD closure, placement of a right-ventricle-to-pulmonary-artery conduit with a polytetrafluoroethylene graft, and pulmonary artery plasty to correct the stenosis at the branch of the pulmonary artery.
Conclusions:
The long-term complications of the Rastelli-type operation seen for D-TGA with a VSD and pulmonary atresia included a right-ventricle-to-pulmonary-artery conduit obstruction, VSD patch leakage, and re-stenosis of the peripheral pulmonary stenosis. Multimodal imaging was informative in planning for reoperation.
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