Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva.

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management.

Current Management of Ebstein's Anomaly in the Adult.

Ebstein's anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein's anomaly.

Anomalous origin of the coronary artery arising from the opposite sinus: prevalence and outcomes in patients undergoing coronary CTA.

Aims: The impact of coronary computed tomographic angiography (CTA) on management of anomalous origin of the coronary artery arising from the opposite sinus (ACAOS) remains uncertain. We examined the prevalence, anatomical characterization, and outcomes of ACAOS patients undergoing CTA.

Methods And Results: Among 5991 patients referred for CTA at two tertiary hospitals between January 2004 and June 2014, we identified 103 patients (1.

Massive Pulmonary Artery Aneurysm Causing Left Main Coronary Artery Compression in the Absence of Pulmonary Hypertension.

We report the case of a 62-year-old woman who presented with classic symptoms of stable angina. Cardiac images and catheterization results revealed absent pulmonary valve syndrome and compression of the left main coronary artery by a massively dilated pulmonary artery aneurysm. The patient's anginal symptoms were relieved after pulmonary arterioplasty.

Usefulness of the Seattle Heart Failure Model to identify adults with congenital heart disease at high risk of poor outcome.

Our objective was to determine whether the Seattle Heart Failure Model (SHFM) differentiates patients with adult congenital heart disease (ACHD) at high versus low risk for cardiovascular outcomes and poor exercise capacity. The ACHD population is growing and presents increasingly for care in the community and at tertiary centers. Few strategies exist to identify the patients with ACHD at high risk for heart failure and mortality.

Adequacy of cancer screening in adult women with congenital heart disease.

Adults with congenital heart disease (ACHD) face noncardiac healthcare challenges as the population ages. We assessed whether women with ACHD have comparable cancer screening rates to non-ACHD women in a cardiac practice and to the general population. We performed a retrospective review of 175 adult women seen in a cardiac care center in 2009-2011.

Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings.

Adult congenital heart disease patients present a unique challenge to the cardiac imager. Patients may present with both acute and chronic manifestations of their complex congenital heart disease and also require surveillance for sequelae of their medical and surgical interventions. Multimodality imaging is often required to clarify their anatomy and physiology.

Quadricuspid aortic valve: a report of 12 cases and a review of the literature.

Quadricuspid aortic valve (QAV) is rare and its diagnosis, clinical course, and management are less well defined relative to other aortic valve abnormalities. Advances in diagnostic imaging, notably in ultrasound, have increased clinical awareness of this anomaly and prompted this review of our experience with 12 new patients and a compilation of previously reported patients to further characterize this condition.

Evaluation of efficacy of 64-slice multidetector computed tomography in patients with congenital coronary fistulas.

Objective: To evaluate the efficacy of 64-slice coronary multidetector computed tomography (MDCT) in the assessment of congenital coronary artery fistula (CCAF).

Methods: Two readers prospectively evaluated the 64-slice coronary MDCT appearances of CCAF in 10 patients (5 men and 5 women; mean age, 58.6 years; range, 19-82 years).

Congenital anomalies of coronary artery origin in adults: 64-MDCT appearance.

Objective: The purpose of this pictorial essay is to review the 64-MDCT appearance of congenital anomalies of the origins of the coronary arteries in adults.

Conclusion: Increasing use of MDCT for cardiac imaging of adults requires familiarity with the cross-sectional appearance of congenital coronary artery anomalies visualized with noninvasive imaging techniques. Many of these anomalies are benign, but a small number are associated with myocardial ischemia and sudden death.

Management of platypnea-orthodeoxia syndrome by transcatheter closure of atrial communication: hemodynamic characteristics, clinical and echocardiographic outcome.

Percutaneous transcatheter closure of patent foramen ovale or atrial septal defect is an alternative to surgery for the treatment of platypnea-orthodeoxia syndrome. We report the hemodynamic characteristics and clinical and echocardiographic outcomes of 18 patients with platypnea-orthodeoxia syndrome that underwent transcatheter closure of patent foramen ovale or atrial septal defect. Successful closure was achieved in all patients with no in-hospital mortality or adverse events.

Survey of specialized tertiary care facilities for adults with congenital heart disease.

Background: Specialized tertiary care facilities developed in response to the increasing numbers of adults with congenital heart disease (CHD). Because this patient population comprises a relatively new area of specialized cardiovascular interest, the first facilities necessarily evolved without preexisting guidelines or interaction.

Objectives: To characterize the major features of the six original and largest tertiary adults CHD facilities.

Prevalence of congenital heart disease.

Background: Today most patients with congenital heart disease survive childhood to be cared for by adult cardiologists. The number of physicians that should be trained to manage these lesions is unknown because we do not know the number of patients.

Methods: To answer this question, the expected numbers of infants with each major type of congenital heart defect born in each 5-year period since 1940 were estimated from birth rates and incidence.

Psychosocial profile of adults with complex congenital heart disease.

Objective: Despite the increasing number of adults with congenital heart disease (CHD), little is known of the emotional life of these long-term survivors; hence, we undertook a study to establish a psychological profile of these individuals so as to optimize their care.

Patients And Methods: We performed psychiatric evaluation by both interview and questionnaire on 29 ambulatory patients with CHD (mean age, 38 years; range, 26-56 years).

Results: Although many met symptomatic criteria for psychiatric diagnosis, most were functional in day-to-day life and used denial in adapting to their CHD.

Combined Rastelli and atrial switch procedure: a 10-year follow-up.

We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.

Arrhythmias in the athlete with congenital heart disease: guidelines for participation.

Advances in the diagnosis and early management of congenital heart disease in recent decades have led to increasing numbers of individuals being sufficiently well to participate in social as well as truly competitive sports. Physicians are increasingly asked whether such participation is safe, advisable, and efficacious, yet few guidelines exist to help them make these decisions. There are three apparent subgroups of patients: (a) those with mild or repaired problems, who function normally or nearly so and may fully participate; (b) those with severe functional deficit or known high risk, for whom strenuous exertion must be strictly proscribed; and (c) those who fall in between, with some limitations and some risk--these patients present a great challenge to the wisdom and clinical skill of the physician.