Isolated ventricular inversion with situs solitus.

The clinical and anatomical findings in two patients with isolated ventricular inversion and situs solitus are described. The other 4 previously published cases are reviewed. The 6 patients with this malformation, all without pulmonary stenosis, presented a clinical picture of cyanotic congenital heart disease, associated with increased pulmonary blood flow (hypoxaemia and cardiac failure). The importance of different diagnostic tests is discussed and it is concluded that angiocardiography is the only definitive means of establishing the dianosis. Because the physiopathological disturbance is the same as in transposition of the great arteries, both malformations should be similarly considered with respect to diagnosis and treatment. Nevertheless, the high incidence of certain associated malformations in cases of isolated ventricular inversion adds to difficulty in diagnosis, and makes a good result from the Mustard procedure less likely than in transposition of the great arteries.     

Isolated ventricular inversion with situs solitus.

The clinical and anatomical findings in two patients with isolated ventricular inversion and situs solitus are described. The other 4 previously published cases are reviewed. The 6 patients with this malformation, all without pulmonary stenosis, presented a clinical picture of cyanotic congenital heart disease, associated with increased pulmonary blood flow (hypoxaemia and cardiac failure). The importance of different diagnostic tests is discussed and it is concluded that angiocardiography is the only definitive means of establishing the dianosis. Because the physiopathological disturbance is the same as in transposition of the great arteries, both malformations should be similarly considered with respect to diagnosis and treatment. Nevertheless, the high incidence of certain associated malformations in cases of isolated ventricular inversion adds to difficulty in diagnosis, and makes a good result from the Mustard procedure less likely than in transposition of the great arteries.     

Parallel arterial trunks with discordant atrioventricular and concordant ventriculo-arterial connections in mirror-imaged arrangement

Cardiac malformations are very rare in which the great arteries arise from their appropriate ventricles, but are abnormally related to each other. Here, we present a patient with mirror-imaged atrial arrangement and a left-sided heart who has parallel arterial trunks with discordant atrioventricular and concordant ventriculo-arterial connections. The ventricles were related in supero-inferior fashion, the left ventricle being anterior and superior, this being an additional feature which, to the best of our knowledge, has not previously been described in this setting.     

Isolated atrial inversion Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: Diagnosis and surgical correction

After cardiac catheterization and angiocardiographic studies an infant with cyanosis from birth was found to have visceral situs solitus, visceroatrial discordance without venoatrial discordance, a discordant ventricular d loop without transposition, and dextrocardia. The circulatory pathway was from the venae cavae to right atrium to left ventricle to aorta, and from the pulmonary veins to left atrium to right ventricle to pulmonary artery. An atrial defect was present allowing some mixing between the pulmonary and systemic circulations, but this appeared inadequate and the defect was enlarged surgically. Subsequently, a corrective operation using an atrial baffle to direct the pulmonary venous return to the left ventricle and the systemic venous return to the right ventricle was successfully undertaken.     

Total correction of double-outlet right ventricle, atrial septal defect, pulmonic stenosis with a concordant atrioventricular relationship, dextrocardia and situs inversus

Surgical correction of a rare type of double-outlet right ventricle was successfully carried out in a 20-year-old man. The malformation was associated with atrial septal defect, ventricular septal defect, pulmonic stenosis, a concordant atrioventricular relationship, and dextrocardia with situs inversus. A Teflon patch was sutured as a baffle in the right ventricle to direct blood that had passed from the left ventricle through the ventricular septal defect out through the aorta. The infundibular obstruction was resected, a pulmonic commissurotomy performed and the atrial septal defect closed. The patient made an uneventful recovery and was well at follow-up 1 year later.     

An adult patient with isolated left ventricular noncompaction presented with atrial fibrillation and complete atrioventricular block

Isolated left ventricular noncompaction (IVNC) is a rare congenital anomaly. The clinical manifestations include congestive heart failure, systemic thromboemboli, arrhythmias and sudden death. We report a case of IVNC in a male patient who presented with atrial fibrillation with complete heart block.     

Mixed levocardia with ventricular inversion (corrected transposition) with complete atrioventricular block A histopathologic study of the conduction system

Serial sections were studied through the conduction system of a heart with mixed levocardia with ventricular inversion, accompanied by atrioventricular block. The normal A-V node ended blindly, while an accessory A-V node gave rise to an accessory A-V bundle and bundle branches. There was interruption by fibrosis and recent hemorrhage of the very tenuous accessory penetrating portion of the A-V bundle.     

Catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with complete situs inversus, atrial septal defect and "inverse" persistent left superior vena cava

Myocardial infarction as a complication of blunt chest trauma has been reported most commonly in victims of car accidents. Other cases have been very rarely reported. To our knowledge, sea traffic accident as the cause of coronary artery injury has not been described. The authors report on a rare case of acute anterior wall myocardial infarction in a 60-year-old woman following blunt chest trauma caused by sea traffic accident.     

Absent right superior caval vein (vena cava) with normal atrial arrangement

Eight cases of absent right superior caval vein (vena cava) with normal atrial arrangement from the Great Ormond Street database were reviewed. In each case the right subclavian vein and jugular vein drained into a persistent left superior caval vein through a bridging vein. In six cases the left superior caval vein drained into the right atrium by way of the coronary sinus, and in two cases it drained into the left atrium directly because of the complete unroofing of the coronary sinus. The frontal plane P wave axis was displaced leftwards and upwards in four out of seven cases in which an electrocardiogram was available. No case showed arrhythmia on a standard electrocardiogram preoperatively. Echocardiograms were available in four cases and in each case diagnosis of the anomalous connections of the systemic vein was possible. Only one third of the cases showed a crescentic shadow of the persistent left superior caval vein on chest x ray. A chest x ray and electrocardiogram do not provide the means of reliable diagnosis of this condition, though they may suggest the possibility of its existence. Definitive diagnosis requires cross sectional echocardiography or angiocardiography or both.     

Absent right superior caval vein (vena cava) with normal atrial arrangement.

Eight cases of absent right superior caval vein (vena cava) with normal atrial arrangement from the Great Ormond Street database were reviewed. In each case the right subclavian vein and jugular vein drained into a persistent left superior caval vein through a bridging vein. In six cases the left superior caval vein drained into the right atrium by way of the coronary sinus, and in two cases it drained into the left atrium directly because of the complete unroofing of the coronary sinus. The frontal plane P wave axis was displaced leftwards and upwards in four out of seven cases in which an electrocardiogram was available. No case showed arrhythmia on a standard electrocardiogram preoperatively. Echocardiograms were available in four cases and in each case diagnosis of the anomalous connections of the systemic vein was possible. Only one third of the cases showed a crescentic shadow of the persistent left superior caval vein on chest x ray. A chest x ray and electrocardiogram do not provide the means of reliable diagnosis of this condition, though they may suggest the possibility of its existence. Definitive diagnosis requires cross sectional echocardiography or angiocardiography or both.     

Anatomic and functional "obstruction" of the outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum atrial septal defect"): an echocardiographic study

Left ventricular (LV) outflow tract (OT) obstruction can be treacherous in any form of atrioventricular (AV) septal defect. The properties of the LVOT were investigated echocardiographically in 64 patients with separate valve orifices ("ostium primum atrial septal defect") who had survived corrective surgery. M-mode and cross-sectional echocardiographic (echo) images were made of the LVOT. The degree of malalignment of the aorta with the ventricular septum, the left atrium-aortic ratio, the fractional LV shortening and the diameter of the LVOT were recorded. Fixed anatomical obstruction was found in 3 patients, consisting of muscular bands or abnormal attachment of tension apparatus. Malalignment of the aorta with the ventricular septum was found in 62% of the patients. The diameter of the LVOT was smaller than that of the aortic root in 71% of the cases. The mean diameter of the LVOT was 92 +/- 27% (range 35 to 143%) of the aortic root diameter. Because its walls are mainly muscular, the LVOT constricts during systole. The mean end-systolic diameter of the LVOT was 77 +/- 22% (range 23 to 129%) of the aortic root diameter. Sequential measurements showed that the LVOT constricted gradually, but the velocity of constriction in patients with the most severe narrowing showed a distinct maximum in the first fifth of systole. In conclusion, a series of elements contribute to a potentially perilous arrangement of the LVOT in patients with AV septal defect. This intrinsically narrow tunnel was constricted during systole by its muscular walls.(ABSTRACT TRUNCATED AT 250 WORDS)     

Apical ballooning by transient left ventricular dysfunction (so-called "ampulla" cardiomyopathy) associated with therapy for acute pulmonary thromboembolism: a case report

An 86-year-old-woman presented with apical ballooning left ventricular dysfunction associated with therapy for acute pulmonary thromboembolism. She was referred to our hospital for advanced treatment for her shock state due to acute pulmonary embolism with normal left ventricular(LV) function. Her condition was stabilized using a percutaneous cardiopulmonary support system. Suction embolectomy was successfully carried out after pulmonary arteriography. After the therapy, echocardiography revealed apical ballooning and hyperkinesis of the base(LV ejection fraction = 28%), although coronary arteriography showed no fixed stenosis. LV wall motion significantly improved on day 3(LV ejection fraction = 45%). Pulmonary embolism relapsed on day 5 in spite of anticoagulation treatment. She died of multiple organ failure on day 9. Autopsy findings indicated no sign of myocardial infarction or myocarditis, patchy appearance of myocardial contraction band necrosis and few migrated lymphocytes. The mechanism for the flow mis-matched LV dysfunction remains unknown. The probable explanations include non-ischemic stress such as catecholamine or neurogenic stress, and possibly ischemic stress or ischemia/reperfusion injury.     

Congenitally corrected transposition in a patient with situs inversus, atrioventricular discordance and mesocardia (I,D,D)

The clinical, hemodynamic and angiographic features of a patient with situs inversus, atrioventricular discordance, corrected transposition of the great arteries (I,D,D) and mesocardia are presented. A review of the literature concerning corrected transposition of the great arteries in situs inversus is included. The incidence of associated cardiovascular and electrocardiographic anomalies is compared with that found in corrected transposition of the great arteries in situs solitus; special surgical aspects of the above cases are also discussed.