Partial Endocardial Cushion Defects

Seven partial endocardial cushion defects have been diagnosed and treated surgically without mortality—six of the ostium primum type and one with a defect from left ventricle to right atrium. The last patient likely has an associated cardiomyopathy. The most serious complication of repair is damage to the bundle of His.The electrocardiogram is helpful in diagnosis, showing left axis deviation in standard leads and right ventricular hypertrophy in chest leads (92%). The vector-cardiogram in the frontal plane shows counterclockwise rotation with the loop usually above the isoelectric line (97%). This is due to congenital aberration of the left bundle branch rather than to left ventricular hypertrophy from mitral incompetence. Radiography demonstrates moderate cardiac enlargement with right-sided hypertrophy, a prominent pulmonary artery, and pleonemic lung fields. Cardiac catheterization reveals a moderate rise in oxygen saturation at atrial level and a further minimal increase at ventricular level. The left atrial trace may show a minimal “c-v” configuration, with the “v” wave equal to the “a”.     

Transcatheter device closure of atrial septal defects in patients aged 40 years and older

Objective. To evaluate the safety and effect of transcatheter device closure in ostium secundum atrial septal defects (ASD II) in patients aged 40 years and older.Methods. Retrospective single-centre study concerning 47 consecutive ASD transcatheter occlusion procedures performed between January 1999 and December 2008. Electrocardiography, echocardiography and clinical assessments of the patients were conducted pre- and post-intervention and at follow-up.Results. Of the 130 patients who were referred for interventional ASD closure, 47 were 40 years and older and all of them actually had the device inserted. There were no major complications during the intervention. Mean follow-up time was 15±15 months. During follow-up, three patients needed surgical reintervention because of device embolisation (n=2) or dislocation (n=1). Of the patients with severe right ventricular (RV) dilatation, more than half (58%) had no or mild dilatation at last follow-up. Reduction of RV dilatation was not related to age. Pulmonary hypertension was present in 63% before the procedure and was reduced to 38% at follow-up. NYHA class improved in all age groups, also in patients over 60 years of age. In two of the three patients who died during follow-up, no cause of death could be established, but both had responded well to treatment regarding the echocardiographic and clinical findings.Conclusion. Transcatheter device closure of ASD is a successful and effective treatment, also for patients aged 40 years and older. Patients showed regression of right ventricular enlargement and an improvement in functional class. (Neth Heart J 2010;18:537–42.)     

Catheter ablation of atrial incisional tachycardia mistaken for atrial flutter

Incisional sustained tachycardias are frequent in patients who have undergone a surgical repair of interatrial defect. A 43-year-old woman with drug refractory, highly symptomatic, persistent atrial tachycardia in the last year, was referred to our unit for catheter ablation. The patient had undergone a cardiac operation for repairing interatrial secundum ostium type defect with a patch five years before. A previous radiofrequency ablation procedure had been performed for common atrial flutter. We describe a case of incisional atrial tachycardia ablation guided by the new EnSite NavX system equipped with a new electroanatomic mapping system.     

Association of cleft lip and atrial septal defect in mice: a preliminary report.

Newborn A/J and CL/Fr mice with congenital cleft lip usually have an atrial septal defect of the secundum type, often associated with cyanosis. Their littermates without cleft lip rarely have a septal defect. The atrial septal defect results from a delay in growth of the atrial septum primum.     

Autosomal dominant atrial septal defect of ostium secundum type. Report of three families

The authors report on three Sicilian families with 17 individuals (10 females and 7 males) in successive generations affected by atrial septal defect of ostium secundum type (S-ASD) without conduction defect. The anomaly was inherited as an autosomal dominant trait. Cytoplasmic inheritance could be excluded, the anomaly being transmitted also by fathers. Familial S-ASD is probably more frequent than commonly reported since cardiological examination of the relatives is not routinely performed in every case of apparently sporadic ASD.     

Reversibility of electrophysiological changes induced by chronic high-altitude hypoxia in adult rat heart

Recent studies indicate that regression of left ventricular hypertrophy normalizes membrane ionic current abnormalities. This work was designed to determine whether regression of right ventricular hypertrophy induced by permanent high-altitude exposure (4,500 m, 20 days) in adult rats also normalizes changes of ventricular myocyte electrophysiology. According to the current data, prolonged action potential, decreased transient outward current density, and increased inward sodium/calcium exchange current density normalized 20 days after the end of altitude exposure, whereas right ventricular hypertrophy evidenced by both the right ventricular weight-to-heart weight ratio and the right ventricular free wall thickness measurement normalized 40 days after the end of altitude exposure. This morphological normalization occurred at both the level of muscular tissue, as shown by the decrease toward control values of some myocyte parameters (perimeter, capacitance, and width), and the level of the interstitial collagenous connective tissue. In the chronic high-altitude hypoxia model, the regression of right ventricular hypertrophy would not be a prerequisite for normalization of ventricular electrophysiological abnormalities.     

The pathogenesis of atrial and atrioventricular septal defects with special emphasis on the role of the dorsal mesenchymal protrusion

Partitioning of the four-chambered heart requires the proper formation, interaction and fusion of several mesenchymal tissues derived from different precursor populations that together form the atrioventricular mesenchymal complex. This includes the major endocardial cushions and the mesenchymal cap of the septum primum, which are of endocardial origin, and the dorsal mesenchymal protrusion (DMP), which is derived from the Second Heart Field. Failure of these structures to develop and/or fully mature results in atrial septal defects (ASDs) and atrioventricular septal defects (AVSD). AVSDs are congenital malformations in which the atria are permitted to communicate due to defective septation between the inferior margin of the septum primum and the atrial surface of the common atrioventricular valve. The clinical presentation of AVSDs is variable and depends on both the size and/or type of defect; less severe defects may be asymptomatic while the most severe defect, if untreated, results in infantile heart failure. For many years, maldevelopment of the endocardial cushions was thought to be the sole etiology of AVSDs. More recent work, however, has demonstrated that perturbation of DMP development also results in AVSD. Here, we discuss in detail the formation of the DMP, its contribution to cardiac septation and describe the morphological features as well as potential etiologies of ASDs and AVSDs.     

The development of the septum primum relative to atrial septation in the mouse heart

The septum primum in the mouse originates as a thickened primordium with a straight rather than a sickle-shaped ventral border. It is covered on its ventral border by anterior cushion material which is continuous over the roof of the atrium with the principal anterior cushion mass. A process of cavitation thins the septum primum and precedes actual fenestration. This process shifts the membranous septum to the left thereby providing room for the septum secundum to overlap on the right side. The septum primum cannot contact the posterior cushion until closure of the sinus venosus gutter which is described. The closure of the interatrial foramen, later the foramen primum, is accomplished by cell growth of the anterior cushion material. The ventral thick border of the septum primum contributes to the ventral limbus and the caudal thickened boundary of the fossa ovalis with some contribution from the left venous valve. These boundaries as well as the membranous portion of the interatrial septum are derived from the same primordium, namely the septum primum.     

Partial trisomy 4(q31qter) due to maternal 4;5 balanced translocation in a neonate

We describe a male neonate with a duplication of 4(q31.3qter) due to unbalanced segregation of a maternal translocation (4;5)(31.3;p15.1). He has a high broad nasal bridge, large, low-set ears, epicanthal folds, long philtrum, retrognathia, high arched palate, wide-spaced nipples, bilateral single transverse palmar creases, bilateral clinodactyly of the fifth finger, right cryptorchidism, and ventricular and secundum type atrial septal defect.     

Rubella Syndrome—Cardiovascular Manifestations and Surgical Therapy in Infants

Of nine patients under five months of age with cardiovascular manifestations of the rubella syndrome, six had patent ductus arteriosus. Three of these six also had pulmonary artery stenosis. One infant had bilateral isolated pulmonary artery stenosis. The significant clinical findings leading to the diagnosis of pulmonary artery stenosis were axillary murmurs in the presence of right ventricular hypertrophy. Demonstration of a gradient across the stenosis at the time of catheterization, together with cineangiography, established the diagnosis. In two cases ventricular septal defect was the only cardiac anomaly.Six babies under five months of age had interruption of a patent ductus arteriosus because of uncontrollable congestive heart failure or failure to thrive. Although growth failure was not necessarily due to heart disease, all were developing satisfactorily following operation.Diagnosis and therapy of the cardiac complications of the rubella syndrome is possible in the first few months of life. Early recognition of cardiac defects in the young infant with the rubella syndrome permits aggressive medical management and in selected instances surgical therapy.     

Transcatheter leadless permanent pacemaker in complex congenital heart disease with interrupted inferior vena cava: A challenging implantation

31 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome, presented with atrial flutter and complete heart block. She was not suitable for corrective cardiac surgery and not yet indicated for heart-lung transplantation. She was advised single chamber permanent pacemaker and eventually Micra VR transcatheter leadless pacemaker was finalised for her. Transcatheter leadless pacemaker was deployed in her RV septum despite some unforeseen technical problems. This patient had intrahepatic interruption of IVC with Azygous continuation draining into SVC but this altered venovascular course was detected only fluoroscopically midway during the pacemaker implantation procedure and this was not detected in the preprocedural transthoracic echocardiography. This abnormal venous course was clearly demonstrated in the cardiac CT which was performed only after completion of the pacemaker implantation procedure in this patient. The technical challenges encountered mainly were mostly during the manipulation of the 27F delivery catheter of Micra through this altered cardiovascular anatomy via transfemoral approach and also due to the presence of septal defects. Thus, transcatheter leadless permanent pacemaker was implanted successfully through transfemoral access in this complex congenital heart disease with interrupted IVC and azygous continuation. Besides transthoracic echocardiography, it may be better to perform transesophageal echocardiography or even preferably radiological imaging like cardiac CT or MRI prior to transcatheter leadless pacemaker implantation in patients with complex congenital heart disease to understand the cardiovascular anatomy and plan the procedure.     

Severe hypoxaemia with a left ventricular assist device in a minipig model with an undiagnosed congenital cardiac disease

We describe the placement of a left ventricular assist device (LVAD) in a pig with spontaneously occurring atrial septal defect (ASD) (incidental finding) that created a right-left cardiac shunt, with subsequent severe hypoxaemia. Early diagnosis was critical in order to prevent end-organ damage due to hypoxaemia. Adequate monitoring alerted us to the deterioration in oxygenation, haemodynamics and cerebral oxygen metabolism. This forced us to change the level of assistance provided by the pump, and thus dramatically correct this impairment. Necropsy revealed an ostium secundum ASD. In conclusion, if hypoxaemia presents after implementation of an LVAD, the presence of a right-left shunt must be ruled out. The first step must be a judicious reduction in assist device flow to minimize intracardiac shunting. Subsequently, atrial septal closure of the defect should be considered. We report an experimental model of severe hypoxaemia after placement of an LVAD as part of a larger research project.     

Radionuclide diagnosis of right ventricular myocardial infarction

The diagnostic capacities of 99mTc-pyrophosphate plane myocardial scintigraphy versus 99mTc-pyrophosphate single photon emission computed tomography (SPECT) were compared. Recording right precordial ECG leads showed that 26 patients had right ventricular myocardial infarction (MI)-typical changes as ST-segment evaluation, followed by abnormal Q wave. Plane scintigraphy indicated a characteristic inclusion of 99mTc-pyrophosphate into the right ventricular myocardium in 18.8% of the patients with acute lower MI and in one of 38 patients with acute MI of the anterior left ventricular wall. SPECT revealed a characteristic inclusion of 99mTc-pyrophosphate into the right ventricular myocardium much more frequently than did plane myocardial scintigraphy--in 34% of cases. Right ventricular myocardial inclusion of 99mTc-pyrophosphate was found in 50% of the patients with acute lower MI, including all 9 patients with positive 99mTc-pyrophosphate myocardial scintigraphy. Thus, the sensitivity of SPECT in the diagnosis of right ventricular MI is somewhat higher than that of precordial ECG and more than thrice higher than that of plane scintigraphy.     

Prevalence of left ventricular hypertrophy in hypertensive and normotensive type 2 diabetic females in Port Harcourt

This study investigated the prevalence of electrocardiographically determined left ventricular hypertrophy in hypertensive and normotensive type 2 diabetic females who went for consultation at the University of Port Harcourt Teaching Hospital (UPTH), Rivers State, Nigeria. Two hundred participants mean age 52years, attending the medical outpatient clinic over a 6-month period were recruited for the study.  Of the population studied, 16.5% of the hypertensive and 13.0% of the normotensive diabetics had left ventricular hypertrophy. Cardiovascular abnormalities notably bifascicular block, left atrial block, right ventricular enlargement, and right atrial enlargement were predominately among hypertensive diabetes and also notably  was arrhythmia  and atrial flutter among normotensive diabetes. The significance of these findings is discussed. Keywords: Hypertension, Diabetes mellitus, Cardiovascular disease, Left ventricular hypertrophy.     

The diagnosis and surgical treatment of tetralogies

Patients with congenital heart disease of the cyanotic type may be presumed to be candidates for surgical treatment if the examination of the heart reveals compatible findings, particularly murmurs characteristic of an interventricular septal defect, overriding aorta, and pulmonary stenosis; if the electrocardiogram shows right axis deviation; if the x-ray or fluoroscopic study demonstrates decreased pulmonary markings; if Diodrast injection shows right ventricular enlargement, a septal defect, overriding of the aorta, and small pulmonary arteries. In some cases some of these criteria may be missing. If there are not definite contraindications, exploratory thoracotomy is indicated for patients with congenital heart disease causing cyanosis.     

Evaluation of late results of surgical treatment of congenital heart disease.

Operative mortality from surgical treatment of congenital heart disease has been steadily reduced over the past 20 years. During the same period it has become clear that a proportion of survivors have residual clinical problems. Some of these are due to imperfect repair, a number being due to factors beyond present surgical control, and some are the consequence of associated cardiovascular defects. The chief problems can be elucidated by an analysis of the results of surgical treatment of six cardiac malformations: simple pulmonary valve stenosis, coarctation of the aorta, secundum atrial septal defect, isolated ventricular septal defect, tetralogy of Fallot and transposition of the great arteries. Recognition of the sequelae of preoperative hemodynamic strain and apparently minor associated malformations is important, and it is possible to anticipate such factors. Long-term follow-up of patients after operation is particularly important.     

Control of myocardial tissue components and cardiocyte organelles in pressure-overload hypertrophy of the cat right ventricle

Previous studies have demonstrated that there is a disproportionate increase in connective tissue in right ventricular myocardium subjected to pressure-overload hypertrophy associated with depressed cardiac contractility. While the myocardium is primarily responsive to load, the aim of the present study was to determine whether catecholamines also modulate the response of myocardial tissue components and cardiocyte organelles in pressure-overload-induced cardiac hypertrophy. Four experimental groups of cats were examined: a sham-operated control group, a group which had their pulmonary arteries banded in order to induce a pressure overload, a group which had been subjected to the same pressure overload, but in addition had beta-adrenoceptor blockade produced prior to and during the pressure overloading, and a group which had been subjected to the same pressure overload, but in addition had alpha-adrenoceptor blockade produced prior to and maintained during the pressure overloading. As in our previous study, there was a significant and equivalent degree of right ventricular hypertrophy in all experimental groups with pressure overload when assessed either as the ratio of right ventricular weight to body weight or as cardiocyte cross-sectional area. At the light microscopic level, the disproportionate increase in the volume density of myocardial connective tissue seen in banded animals was completely prevented by either alpha- or beta-adrenoceptor blockade. At the electron microscopic level, there was a reduction in the mitochondrial and myofibrillar volume fractions following beta-adrenoceptor blockade. The results of this study provide evidence for a modulatory role of catecholamines in the control of myocardial connective-tissue proliferation in pressure-overload-induced cardiac hypertrophy. There is also evidence to support the role of the adrenergic nervous system in regulating cardiocyte subcellular organelles, independent of the regulation of cardiocyte size.     

PRENATAL CARE

Patients with congenital heart disease of the cyanotic type may be presumed to be candidates for surgical treatment if the examination of the heart reveals compatible findings, particularly murmurs characteristic of an interventricular septal defect, overriding aorta, and pulmonary stenosis; if the electrocardiogram shows right axis deviation; if the x-ray or fluoroscopic study demonstrates decreased pulmonary markings; if Diodrast injection shows right ventricular enlargement, a septal defect, overriding of the aorta, and small pulmonary arteries.In some cases some of these criteria may be missing. If there are not definite contraindications, exploratory thoracotomy is indicated for patients with congenital heart disease causing cyanosis.