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Congenital extracardiac shunts in tetralogy of Fallot may significantly alter the history and clinical findings and also have significant management implications. An unusual variant of severe tetralogy of Fallot in a 54-year-old man enabled long survival with minimal symptoms. The patient had pulmonary valvular and infundibular stenosis, a large ventricular septal defect, an overriding aorta, and large volume systemic-to-pulmonary artery shunting from his right internal mammary and accessory internal mammary arteries to his right pulmonary artery. [ABSTRACT FROM AUTHOR]

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 (1.7% prevalence) with 110 ACAOS vessels. Mean age was 52 years (range 5-83, 63% male), with 55% previously known ACAOS and 45% discovered on CTA. ACAOS subtypes included: 39% interarterial (n = 40 anomalous right coronary artery, n = 3 anomalous left coronary artery), 38% retroaortic, 15% subpulmonic, 5% prepulmonic, and 2% other. ACAOS patients were assessed for symptoms, ischaemic test results, revascularization, all-cause or cardiovascular (CV) death, and myocardial infarction. CTAs were reviewed for ACAOS course, take-off height and angle, length and severity of proximal narrowing, intramural course, and obstructive coronary artery disease (CAD). In follow-up (median 5.8 years), there were 20 surgical revascularizations and 3 CV deaths. After adjusting for obstructive CAD (n = 21/103, 20%), variables associated with ACAOS revascularization included the following: CV symptoms, proximal vessel narrowing ≥50%, length of narrowing >5.4 mm, and an interarterial course., Conclusion: The prevalence of ACAOS on CTA was 1.7%, including 45% of cases discovered incidentally. CTA provided excellent characterization of ACAOS features associated with coronary revascularization, including the length and severity of proximal vessel narrowing., (Published by Oxford University Press on behalf of the European Society of Cardiology 2016. This work is written by US Government employees and is in the public domain in the US.)

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. Others have described proximal left main coronary artery compression in the presence of a dilated and hypertensive pulmonary artery. To our knowledge, this is the first case in which a pulmonary artery aneurysm caused left main coronary insufficiency in the absence of pulmonary hypertension-a clinically important complication of congenital pulmonary valve-related pulmonary arteriopathy.

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.We studied 153 adults with transposition of the great arteries, Ebstein anomaly, tetralogy of Fallot, double outlet right ventricle, and single ventricle from 2 ACHD centers. The primary outcome was cardiovascular death, with a secondary composite outcome of death, transplant, ventricular assist device, cardiovascular admission, and treatment for arrhythmia. We defined risk groups based on SHFM 5-year predicted survival: high (predicted survival <70%), intermediate (70% to 85%), and low risk (>85%). Ten patients had the primary outcome of death, and 46 the combined end point. The hazard of death in the SHFM high- versus the intermediate-risk group was 7.09 (95% confidence interval 1.5 to 33.4, p = 0.01; no deaths in the low-risk group) and the hazard of the composite outcome between the high- versus low-risk group was 6.64 (95% confidence interval 2.5 to 17.6, p = 0.0001). Kaplan-Meier survival analysis showed greater probability of all-cause mortality (p = 0.003) in the high-risk group. In conclusion, the SHFM can help identify subjects with ACHD at risk for adverse outcome and poor cardiopulmonary efficiency. This may add to the care of patients with ACHD in the community and streamline care at tertiary centers., (Copyright © 2014 Elsevier Inc. All rights reserved.)

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. Radiation dose is of particular concern in these patients with lifelong imaging needs for their chronic disease. The second-generation dual-source scanner is a recently available advanced clinical cardiac computed tomography (CT) scanner. It offers a combination of the high-spatial resolution of modern CT, the high-temporal resolution of dual-source technology, and the wide z-axis coverage of modern cone-beam geometry CT scanners. These advances in technology allow novel protocols that markedly reduce scan time, significantly reduce radiation exposure, and expand the physiologic imaging capabilities of cardiac CT. We present a case series of complicated adult congenital heart disease patients imaged by the second-generation dual-source CT scanner with extremely low-radiation doses and excellent image quality., (© 2012 Wiley Periodicals, Inc.)

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., (© 2011, Wiley Periodicals, Inc.)

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. Complete resolution of symptoms was seen in all patients and mean oxygen saturation increased from 82.6 +/- 5.4% to 96.1+/-2.2% post-procedure (p < 0.01).

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., Methods: Written questionnaire sent to six participating facilities in North America and Europe. Information was analyzed centrally., Results: All but one facilities was established over 20 years ago, and each cares for over 1500 patients. Hospital admissions ranged from 100 to 660 patients/unit/year. Of the total number of registered patients, 52-81% had undergone one or more reparative surgeries. Reoperations constituted 25-80% of the 50-170 operations/unit/year. Overall mean surgical mortality was 1.9%/year. Inpatient and outpatient care was provided in adult (n = 4) or both adult and pediatric (n = 2) settings. All six facilities enjoyed close collaboration between adult and pediatric cardiologists, cardiac surgeons, nurse specialists and cardiac and non-cardiac consultants. Training and research were pivotal activities., Conclusions: Provision of comprehensive care by multidisciplinary teams including adult and pediatric cardiologists, cardiac surgeons, specialized nurses and other cardiac and non-cardiac consultants was the unifying feature for all six tertiary care facilities reported here. There were minor differences among them based on available resources, local expertise and national health care policies. There appears to be a significant shortfall in tertiary care provision for the adult with CHD that requires further planning and resource allocation. These data may be useful for new and evolving adult CHD services., (Copyright 2003 Elsevier Ireland Ltd.)

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. In childhood, extended absences from school, cyanosis, scars, and sports restrictions hindered friendships and conflicted with a sense of "normalcy"; teasing and low self-esteem were more notable in boys; girls more readily hid behind makeup, nail polish, and clothing. In adulthood most were concerned about shortened life expectancy, disability, and childbearing and child rearing. Virtually all were highly committed to both school and work performance. All but those with complete, uncomplicated early repair expressed unresolved frustration, anger, and fears of a future that might include further medical complications., Conclusion: Listening to this selected group of patients offers insight that may be helpful to the clinical care of patients with CHD and other patients with lifelong disease.

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.

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.

Athletes are subject to the same arrhythmias as the general population, but the frequency and significance of the arrhythmias may be different. Cardiovascular conditioning slows the heart rate and may make athletes more vulnerable to neurocardiogenic syncope and atrial fibrillation. Tachyarrhythmias may be precipitated by vigorous exercise and more severe rate-related symptoms may result because of the high sympathetic drive during sports activities. For those with pre-existing cardiovascular abnormalities, athletic activity may be beneficial in some cases, but dangerous and even life-threatening in others. A review of the subject and recommendations based on our personal experience and a recent consensus conference are provided.

Background: Development of techniques for percutaneous closure of atrial septal defects (ASDs) makes accurate noninvasive sizing of ASDs important for appropriate patient selection., Methods and Results: Magnetic resonance (MR) images of ASDs were obtained in 30 patients (mean age, 41 +/- 16 years) by both spin-echo and phase-contrast cine MR imaging. Spin-echo images were obtained in two orthogonal views (short-axis and four-chamber) perpendicular to the plane of the ASD. Spin-echo major and minor diameters were measured, and spin-echo defect area was calculated. Phase-contrast cine MR images were obtained in the plane of the ASD, and cine major diameter and defect area were measured from the region of signal enhancement or phase change due to shunt flow across the defect. MR measurements were compared with templates cut during surgery to match the defect or with ASD diameter determined by balloon sizing at catheterization. ASD size measured from cine MR images (y) agreed closely with catheterization and template standards (x). For major diameter, y = 0.78x + 5.7, r = .93, and SEE = 3.4 mm. On average, spin-echo measurements overestimated major diameter and area of secundum ASDs by 48% and 125%, respectively., Conclusions: Phase-contrast cine MR images acquired in the plane of an ASD define the defect shape by the cross section of the shunt flow stream and allow noninvasive determination of defect size with sufficient accuracy to permit stratification of patients to closure of the defect by catheter-based techniques versus surgery. Spin-echo images, on the other hand, are not adequate for defining ASD size, because septal thinning adjacent to a secundum ASD may appear to be part of the defect.

Of 131 patients with panic disorder or agoraphobia, 44 (34%) had definite mitral valve prolapse on the basis of clinical findings of an apical mid- or late systolic click and a murmur and/or a two-dimensional echocardiogram showing prolapse of one leaflet in two views or of two leaflets in a single view. Seven other patients (5%) had a probable diagnosis of mitral prolapse on the basis of a typical murmur alone or an intermittent apical mid- or late systolic click and a prolapsing leaflet in a single echocardiographic view. This finding confirms previous reports of the association between panic disorder and mitral valve prolapse.

To clarify the clinical spectrum of coronary arterial abnormalities in systemic lupus erythematosus, the data were reviewed on six patients who had a diagnosis of lupus at ages 15 to 29 years and who had ischemic heart disease before age 35. Two patients had coronary arteritis diagnosed on postmortem examination. In a third patient alterations in coronary arterial anatomy occurred with angiographic improvement temporally related to the initiation of steroid therapy. The other three patients had severe diffuse atherosclerotic coronary disease that was identified in two at postmortem examination. In the third patient the course of the disease strongly suggested coronary atherosclerosis, and eventually coronary bypass grafting was performed for relief of angina. In summary, clinically important extramural coronary arteritis and atherosclerosis both occur, although rarely, in young patients with lupus. Coronary artery disease may occur with or without coexisting active extracardiac lupus manifestations. Short-term steroid therapy and follow-up angiography for those with angina and in whom coronary arteritis is suspected warrant consideration. When stable coronary arterial anatomy is demonstrated on follow-up angiography, management is determined by the patient's symptoms irrespective of the prior history of lupus and, if indicated, cardiac surgery for symptomatic relief can be safely performed.

Causative factors, clinical consequences and treatment of atrial tachyarrhythmias were reviewed in 917 monitored patients with definite acute myocardial infarction. Significant atrial tachyarrhythmias were found in 104 (11 per cent) of them and included atrial fibrillation in 67, atrial flutter in 29 and paroxysmal atrial tachycardia in 33. These episodes were single in 79 patients and multiple in 25, and began within the first four days of acute myocardial infarction in 90 per cent of the patients. Fifty per cent of these atrial tachyarrhythmias were heralded by premature atrial contractions. The incidence of atrial tachyarrhythmia was not related to the location of the acute myocardial infarction or to the presence or degree of power failure; however, atrial tachyarrhythmias were significantly more frequent in patients with pericarditis. Atrial tachyarrhythmias were well tolerated in almost one fifth of the patients, caused marginal compromise in almost two thirds and led to severe clinical deterioration in one fifth. Paroxysmal atrial tachycardia rarely required specific treatment, atrial fibrillation was best managed with intravenous administration of digoxin except when associated with severe clinical compromise, and atrial flutter generally required cardioversion or rapid intravenous therapy and usually caused severe clinical deterioration. Over-all, atrial tachyarrhythmia was not associated with a significantly increased mortality, and in those who died, death was not related specifically to the atrial tachyarrhythmia but rather to the severity of the underlying acute myocardial infarction. However, persisting atrial tachyarrhythmias, particularly atrial flutter which tends to be refractory to both heart rate control and cardioversion, may contribute indirectly to morbidity and mortality.

We investigated the function of the conus arteriosus in the bullfrog Rana catesbeiana using a combination of anatomical and physiological techniques. Although there is a normal delay in ventriculoconal conduction and we could induce a spectrum of ventriculoconal conduction disturbances by manipulating the region of the ventriculoconal junction, we found no histological evidence of specialized conducting myocardial tissue in this region. The performance of the conus arteriosus was explored during various disturbances of ventriculoconal conduction and also during hemodynamic disturbances produced by hemorrhage and afterloading. The conus was found to contribute little to forward flow under ordinary circumstances, but its contribution increased greatly during bleeding or partial occlusion of the truncus. In contrast to the conclusion of others, no evidence could be adduced to support the idea that the conus serves as a depulsating chamber. Disparities in previous reports concerning the operation of the conus as a booster pump are attributed to special experimental circumstances.