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Congenital anomalies of the heart can pose challenges to cardiac invasive procedures. Here, we present the case of a 40-year-old man with the combination of dextrocardia, a persistent left superior vena cava, and idiopathic dilated cardiomyopathy. We describe the successful implantation of a biventricular pacemaker-defibrillator under this complex anatomic condition.
This article reports on the transhepatic closure of post-Fontan hepatic veins to left atrium fenestration in a 13-year-old girl with left atrial isomerism, dextrocardia, azygos continuation to the left superior vena cava, and median liver. The transhepatic route seemed to be the less tortuous access to the fenestration which was previously partially occluded with a CardioSeal device. The hepatic veins were entered at the left abdominal side using fluoroscopic guidance and the fenestration was uneventfully occluded with an Amplatzer septal occluder device.
Tricuspid and aortic valve replacement was performed on a 50-year old man with dextrocardia, situs solitus, D-ventricular looping, and normally related great arteries. Twenty-two years earlier the patient had undergone repair of an incomplete atrioventricu lar canal and mitral valve replacement. The anatomic issues and operative considerations are discussed for aortic and tricuspid valve replacement in this anatomic situation with special emphasis on tricuspid valve replacement through a right ventriculotomy. Six months after surgery, New York Heart Association functional class improved from class III preoperatively, to class II.
The authors report a case of a 39-year-old woman with dextrocardia and situs inversus who presented with episodes of complete heart block, managed successfully with a permanent dual chamber endocardial pacemaker.
Dextrocardia can be defined as a heart in the right chest with the major axis to the right. This definition, however, conveys no information regarding the chamber arrangements and internal anatomy of the heart. Of 40 patients satisfying this definition in the files of the Brompton Hospital, 33 had angiocardiographic data adequate for complete analysis in terms of connections, relations, and morphology of cardiac segments. They form the subject of this report. There were 16 (48%) patients with situs solitus, 11 (33%) with situs inversus, and six (18%) with situs ambiguus. Of the cases of situs ambiguus, four exhibited laevoisomerism and two dextroisomerism. Of the 16 patients with situs solitus, six had two ventricles and 10 had univentricular hearts; two patients had concordant and three discordant ventriculoarterial connections, seven had double outlet ventricle, and four a single outlet heart. Of the 11 patients with situs inversus, nine had two ventricles and two a univentricular heart of right ventricular type; the arterial connection was concordant in two, discordant in two, double outlet in six, and single outlet in one. Of the six patients with situs ambiguus and laevo or dextroisomerism, four had two ventricles, and two univentricular hearts; the arterial connection was concordant in one, double outlet in three, and single outlet in two. Segmental analysis and the use of basic descriptive terms are essential to define the complex anatomy of such hearts.
In three cases of dextrocardia with situs inversus affecting a father and his two sons, the father had married his first cousin, the daughter of his father's sister, so that the two sons were the products of a consanguineous marriage. The general and familial incidence of the condition and the genetic influences responsible for it are discussed.
We report a female baby with multiple congenital anomalies including left upper amelia, congenital short bowel with malrotation and pseudo-obstruction, dextrocardia with situs solitus, patent ductus arteriosus, and a tiny atrophic spleen. Chromosome study showed de novo 46,XX/46,XX,-4, + r(4)(p16-->q22.3)/47,XX,4, + r(4) (p16-->q22.3), + del(4)(pter-->q22.3:). The clinical findings in the patient were probably caused by the interaction of partial trisomy 4pter-->q22.3 or 4p16-->q22.3 and partial monosomy of 4q22.3-->4qter. This karyotype and phenotype have not previously been reported.
A case of complete oesophageal duplication in association with dextrocardia and situs inversus is presented.
We report here a case of dextrocardia with normal situs in an 81-year-old man who died from non-pulmonary causes. Removal of the chest wall revealed a large, but otherwise anatomically normal, left lung occupying the entire left hemithorax and extending across the midline to overlap the left border of the heart which was positioned in the right hemithorax. The gross anatomy of the heart was normal save its position and the presence of only 2 pulmonary veins. Dissection of the heart showed all chambers in their classically described position, and there were no valvular defects. The anatomy of the great vessels was also normal. The right lung was hypoplastic and lay posterior to the heart. This lung lacked any lobular structure, but the presence of carbon particles throughout it suggested that it was capable of normal inflation.
Variants of classical exstrophy are exceedingly rare. We report a case of covered and duplicate exstrophy with duplication of the bladder, urethra and genital tract with dextrocardia.
A patient with dextrocardia who suffered his first myocardial infarction after approximately 26 hours of a diagnostic sleep deprivation protocol is described. The infarction started about 3 hours after a significant improvement in mood, which persisted during and after infarction. Total sleep deprivation may be an acute risk factor for myocardial infarction.
A sixty-year-old man who presented with dyspnea and palpitations was investigated in this institution and was found to have dextrocardia, single aortic coronary ostium, severe aortic regurgitation, and atrioventricular nodal reentrant tachycardia. The association of single aortic coronary ostium and this combination of cardiac abnormalities has not been previously reported.
Four cases of successful coronary angioplasty for anomalous coronary arteries, including dextrocardia associated with three-vessel disease, single left coronary artery with proximal left anterior descending lesion, anomalous right coronary artery (RCA) from adjacent left coronary sinus of Valsalva associated with proximal RCA lesion, and anomalous left circumflex angulated lesion bifurcated from the RCA, were encountered. Four cases with 8 target lesions who had a mean age of 63.5 +/- 11.5 years old are presented. All the targets lesions were completely dilated through balloon angioplasty, including use of a newly developed support device for cases with large jeopardized myocardium. The factors for complete revascularization were appropriate selection of catheters and originality and ingenuity of procedural technique based on the anatomic characteristics.
The authors report a case of a forty-one-year-old white woman with dextrocardia with situs inversus who presented episodes of prolonged sinus arrest and syncopal episodes secondary to possible idiopathic degeneration of the conduction system, managed successfully with a permanent bicameral pacemaker. In their literature review they found that this case represents a very rare association.
A 42-year-old man with mirror-image dextrocardia was found to have idiopathic hypertrophic subaortic stenosis. The coexistence of these uncommon congenital cardiovascular anomalies was previously reported in only one patient.
A case of inferior wall myocardial infarction in a patient with mirror-image dextrocardia and situs inversus totalis is presented. The clinical and electrocardiographic findings are discussed.
A patient with the sick sinus syndrome accompanying mirror-image dextrocardia which was associated with double superior venae cavae and an absent inferior vena cava is presented. A permanent transvenous demand pacemaker was inserted because of repeated episodes of dizziness and a documented 31/2-second period of asystole. Precise knowledge of the venous system and the location of the apex of the right ventricle was necessary prior to permanent pacemaker implantation.
Two case reports are described to illustrate the unusual occurrence of dominant inheritance of Peters' anomaly and the concomitant occurrence of Peters' anomaly with colobomatous microphthalmos and dextrocardia. Studies of additional families are necessary to determine conclusively the pathogenesis, genetic mode of inheritance, ocular and systemic associated malformations, and proper management of this complex entity.