Abnormal origin of right pulmonary artery from the ascending aorta in an infant ("Hemitruncus").

Key Clinical Message: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital malformation and it needs surgical management. Consequences of this condition affect lead to pulmonary hypertension and severe pulmonary vascular disease. Abstract: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality affecting the right pulmonary artery more than the left. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative.

Inappropriate shocks from a transvenous implantable defibrillator caused by atrial fibrillation and a missed atrial septal defect in a patient with a modified Bentall procedure.

BACKGROUND: Inappropriate shocks have been reported in ∼1/3 of patients with implantable cardiac defibrillators (ICDs). We report an unusual case of inappropriate ICD shocks due to atrial fibrillation (AF) caused by a missed atrial septal defect (ASD) in a patient with a modified Bentall procedure. CASE SUMMARY: A 67-year-old Caucasian male, with an ICD and a history of a modified Bentall procedure 24 years ago, reported to our outpatient clinic with recurrent inappropriate ICD shocks due to episodes of fast AF. The transthoracic echocardiographic exam revealed two large aneurysms at the ostia of the coronary arteries. We performed further evaluation with transoesophageal echocardiogram and computed tomography (CT) angiography. The aneurysms measured on CT were 3.14 cm × 2.29 cm on the right ostium and 1.9 cm × 0.99 cm on the left. A large secundum type ASD of 1.5 cm, missed in all previous echocardiographic studies, was revealed. The therapeutic options of surgical closure of the ASD and repair of the aneurysms or a more conservative approach with percutaneous closure of the ASD and closer follow-up were discussed with the patient. The patient declined the surgical option due to high complication risk, and closure of the ASD with an Amplatzer device was performed 3 months later. A 3-year follow-up was uneventful. CONCLUSION: It is of major importance to comprehensively and thoroughly assess patients before and after a surgical intervention to not miss other treatable conditions preoperatively and complications in the postoperative period.

Silent Myocardial Perfusion Abnormalities Detected by Stress Cardiovascular Magnetic Resonance in Antiphospholipid Syndrome: A Case-Control Study.

Objective: To examine the prevalence of silent myocardial ischemia and fibrosis in antiphospholipid syndrome (APS), using stress cardiovascular magnetic resonance (CMR). Methods: Forty-four consecutive APS patients without prior cardiac disease (22 primary APS, 22 systemic lupus erythematosus (SLE)/APS, mean age 44 (12.9) years, 64% women) and 44 age/gender-matched controls were evaluated using CMR at 1.5 T. Steady-state free precession imaging for function assessment and adenosine stress-CMR for perfusion-fibrosis evaluation were employed. The myocardial perfusion reserve index (MPRI), and myocardial fibrosis expressed as late gadolinium enhancement (LGE), were evaluated. Coronary angiography was indicated in patients with LGE. Associations with APS characteristics, classic cardiovascular disease (CVD) risk factors, high-sensitivity CRP (hs-CRP) and high-sensitivity Troponin (hs-TnT) levels were tested. All patients were followed up for 12 months. Results: Median MPRI was significantly lower in APS patients versus controls [1.5 (0.9-1.9) vs. 2.7 (2.2-3.2), p < 0.001], independently of any LGE presence. LGE was detected in 16 (36.3%) patients versus none of controls (p < 0.001); 12/16 were subsequently examined with coronary angiography and only two of them had coronary artery lesions. In multivariable analysis, none of the APS-related and classic CVD risk factors, or hs-CRP and hs-TnT covariates, were significant predictors of abnormal MPRI or LGE. At the twelve month follow-up, three (6.8%) patients experienced coronary artery disease, notably those with the lowest MPRI values. Conclusions: Abnormal MPRI and LGE are common in asymptomatic APS patients, independently so of any APS-related and classic CVD risk factors, or coronary angiography findings in cases with LGE. Stress-CMR is a valuable tool to detect silent myocardial ischemia and fibrosis in APS.

Rare multiple bronchial abnormalities in a patient with congenital heart disease.

Although many variations regarding lobar or segmental bronchial subdivisions have been described, abnormal bronchi originating from the trachea or main bronchi are relatively rare. These abnormalities can remain undetectable as they usually do not present with symptoms; however, they may pose major obstacles during surgery especially when accompanied by bronchial wall abnormalities.

Growth rate of an apical left ventricular myxoma using serial two dimensional echocardiographic and computed tomography observations over twelve months: a case report.

INTRODUCTION: Myxomas are the most common benign tumors of the heart. They vary widely in size, and little is known about their growth rate. The present case report is, we believe the first in bibliography that provides images of an apical left ventricular myxoma from transthoracic echocardiography and computed tomography scans taken a year apart. CASE PRESENTATION: We present the rare case of a 65-year-old asymptomatic Caucasian man with an apical left ventricular myxoma that grew over a 12-month period. Our patient underwent successful surgical excision of the tumor and had an uneventful postsurgical recovery period. CONCLUSIONS: Left ventricular myxomas are benign and curable tumors. They do not usually present with systemic symptomatology and most of them are diagnosed as sequelae of syncope caused by left ventricular outflow tract obstruction or systemic embolization. Because they are usually removed after diagnosis, the growth rate of myxomas is generally unknown. We present a rare case of the asymptomatic presentation of a left ventricular myxoma and its documented growth during a 12-month period.