Cyanotic congenital heart disease the coronary arterial circulation.

Background: The coronary circulation in cyanotic congenital heart disease (CCHD) includes the extramural coronary arteries, basal coronary blood flow, flow reserve, the coronary microcirculation, and coronary atherogenesis.

Methods: Coronary arteriograms were analyzed in 59 adults with CCHD. Dilated extramural coronaries were examined histologically in six patients.

The cardiac malpositions.

Dextrocardia was known in the 17th century and was 1 of the first congenital malformations of the heart to be recognized. Fifty years elapsed before Matthew Baillie published his account of complete transposition in a human of the thoracic and abdominal viscera to the opposite side from what is natural. In 1858, Thomas Peacock stated that "the heart may be congenitally misplaced in various ways, occupying either an unusual position within the thorax, or being situated external to that cavity.

The variant associations of aortic isthmic coarctation.

The term "coarctation" necessarily calls attention to a specific morphologic abnormality of the aortic isthmus. However, in this report, the author seeks to dispel the simplistic notion that coarctation is best characterized by isthmic obstruction, which is only 1 of an assemblage of abnormalities that include the proximal paracoarctation aorta, the distal paracoarctation aorta, the ascending aorta, the transverse aorta, the coronary arteries, the conduit arteries (radial, brachial, and carotid), the retinal vascular bed, dissecting aneurysms, cerebral aneurysms, vascular rings, systemic hypertension, and a decrease in left ventricular interpapillary muscle distance. Some of these abnormalities are secondary to the coarctation, such as collateral arteries and dissecting aneurysms.

Retinal vascular patterns after operative repair of aortic isthmic coarctation.

The retinal arteriolar abnormality that accompanies aortic isthmic coarctation is as distinctive as it is unique but has thus far been virtually ignored. Accordingly, 10 patients (4 men and 6 women) were recruited for a study from the Ahmanson/UCLA Adult Congenital Heart Disease Center Outpatient Clinic. Patient age at enrollment was 35 + or - 4 years (range 25 to 66).

Retinal vascular patterns in adults with cyanotic congenital heart disease.

Introduction: The purpose of this report is to describe retinal findings in adults with cyanotic congenital heart disease.

Methods: Four patients, 27 to 47 years of age, with cyanotic congenital heart disease were enrolled from the Outpatient Clinic of the Ahmanson/UCLA Adult Congenital Heart Disease Center. Each patient had a medical history and physical examination by a cardiologist, chest X-ray, electrocardiogram, echocardiogram, complete blood count, and arterial oxygen saturation.

Prevalence of migraine headaches in patients with congenital heart disease.

The prevalence of migraine headaches (MH) is 12% in the general population and increases to 40% in patients with patent foramen ovale. This study evaluated the prevalence of MH in patients with congenital heart disease (CHD). Of 466 patients contacted from the UCLA Adult Congenital Heart Disease Center, 395 (85%) completed a questionnaire to determine the prevalence of MH.

Nitric oxide metabolism in adults with cyanotic congenital heart disease.

Endothelial-derived nitric oxide (NO) diffuses abluminally to regulate blood flow by activating soluble guanylate cyclase in medial smooth muscle. However, a significant fraction of NO diffuses luminally, where the extremely high reaction rate with red blood cell hemoglobin (Hb) effectively reduces luminal concentration to zero. The erythrocytosis of cyanotic congenital heart disease has potentially opposing effects, namely, a reduction in medial smooth muscle NO bioavailability because of the increase in luminal consumption of the molecule and, conversely, an increase in the elaboration of NO in response to the high endothelial shear stress of the erythrocytotic perfusate.

Usefulness of post-ventriculotomy signal averaged electrocardiograms in congenital heart disease.

Signal-averaged electrocardiography is a sensitive and specific technique for detecting the slow conduction electrophysiologic substrates of reentrant monomorphic ventricular tachycardia. Although well established, the method has not been used for electrophysiologic assessment after right ventriculotomy for the intracardiac repair of congenital heart disease. This 8-year prospective study provided this assessment.

The coronary microcirculation in cyanotic congenital heart disease.

Background: Despite an appreciable increase in basal coronary blood flow in cyanotic congenital heart disease, flow reserve remains normal. We hypothesized that preservation of flow reserve resides in remodeling of the coronary microcirculation. Microcirculatory morphometric analyses were performed to test this hypothesis.

Pathogenesis of thrombocytopenia in cyanotic congenital heart disease.

Although a significant minority of patients with cyanotic congenital heart disease (CCHD) are thrombocytopenic, the pathogenesis and prevalence have not been established. This study was designed to address these 2 issues. We included 105 patients with CCHD (60 men and 45 women; aged 21 to 54 years).

Pulmonary neovascularity: a distinctive radiographic finding in Eisenmenger syndrome.

Background: We sought to characterize the distinctive pulmonary vascular abnormalities seen on chest radiographs and computed tomography (CT) scans in Eisenmenger syndrome.

Methods And Results: Thoracic CT scans, chest radiographs, and clinical data were reviewed for 24 Eisenmenger syndrome patients subdivided into those with interatrial (pretricuspid) versus interventricular or great arterial (posttricuspid) communications and in 14 acyanotic patients with pulmonary arterial hypertension (PAH) and no congenital heart disease. CT scans were scored blindly by 2 thoracic radiologists for the presence and severity of small, tortuous, intrapulmonary vessels, termed "neovascularity," lobular ground-glass opacification, and systemic perihilar and intercostal vessels.

Cyanotic congenital heart disease and coronary artery atherogenesis.

Hypoxemic erythrocytotic residents of high altitudes lack coronary atherosclerosis and have low cholesterol levels. It was postulated that hypoxemic erythrocytotic adults with cyanotic congenital heart disease (CCHD) might be analogous. The incidence of coronary atherosclerosis in this patient population has not been established, and hypocholesterolemia has not previously been recognized.

Myocardial perfusion reserve in adults with cyanotic congenital heart disease.

In patients with cyanotic congenital heart disease (CCHD), a right-to-left shunt results in systemic hypoxemia. Systemic hypoxemia incites a compensatory erythrocytosis, which increases whole blood viscosity. We considered that these changes might adversely influence myocardial perfusion in CCHD patients.

Gynecological health of females with congenital heart disease.

Because of major advances in diagnostic and surgical methods, females with congenital heart disease (CHD) now survive into and beyond their reproductive years. Management of pregnancy in this patient population is well described, but gynecologic management such as menstruation, contraception and menopause have received scanty attention. Accordingly, the gynecologic health issues confronting these patients are described.

The coronary circulation in cyanotic congenital heart disease.

Background: Dilated coronary arteries, coronary blood flow, and the paucity of coronary atherosclerosis have not been studied in cyanotic congenital heart disease.

Methods: Coronary arteriograms were interpreted in 59 cyanotic adults, and dilated coronaries were examined histologically in 6. Coronary blood flow was determined with N-13 ammonia positron emission tomography in 14 Eisenmenger syndrome patients and in 10 controls.

Extramural coronary arteries in adults with cyanotic congenital heart disease.

Dilatation and tortuosity of extramural coronary arteries are prevalent in cyanotic congenital heart disease. Two pathogenetic variables are operative, namely endothelial vasodilator substances and medial structural abnormalities.

Surgical treatment of patent ductus arteriosus: a new historical perspective.

The conventional surgical history of ligation of a patent ductus arteriosus (PDA) dates from August 26, 1938, when Robert E. Gross of Boston, Massachusetts, successfully ligated a PDA. It is largely unknown that in the same year and before Gross, Emil Karl Frey, a Surgeon at the Medizinische Akademie in Dusseldorf, Germany, already ligated a PDA successfully.

The Howard Gilman Foundation Lecture. Where have we come from and where are we going? Valve management past, present and future.

Leonardo da Vinci's anatomical drawings of quadricuspid, tricuspid, and bicuspid aortic valves underscored the hydraulic superiority of a three leaflet valve with cuspal equality. William Harvey demonstrated that venous valves were designed for unidirectional flow and to prevent reflux from the heart, observations that served as the basis of his immortal de Mortu Cordis. Joseph Rouanet of Paris proposed that heart sounds originated from the closing movements of cardiac valves.

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.