Aneurysms of the sinus of Valsalva are extremely rare. Ruptured aneurysms of the sinus of Valsalva are frequently associated with other congenital defects, particularly with ventricular septal defect, aortic valve regurgitation, and bicuspid aortic valve. We describe the case of a 26-year-old man who had a ruptured aneurysm of the ...

Cyanosis in atrial septal defect typically occurs when pulmonary hypertension develops. Platypnea-orthodeoxya is an uncommon syndrome, still under debate, characterized by breathlessness and arterial oxygen desaturation exacerbated in the upright position. An interatrial communication is a common finding in this syndrome, but the absence of a right to left pressure ...

A 4-hour-old boy is presented here, who was born with a large abdominal wall defect situated in the left flank. Silo closure was necessary due to the large size of the defect. A Medline search up to December 2002 revealed only 2 other left-sided abdominal wall defects distant from the ...

A 52-year-old Argentinian woman presented with third-degree AV block due to seropositive chronic stage of Chagas' disease. Subsequently, a DDD pacemaker was implanted. Interestingly, a postoperative chest X ray suggested left ventricular lead misplacement, an ECG showed a paced RBBB. Echocardiography confirmed suspected lead malposition in the left ventricle with ...

The present study shows that in about 12.4% of subjects the first septal artery (FSA) is very near from the His bundle. Routine measurement of His-FSA distance during percutaneous transluminal septal myocardial ablation (PTSMA) procedure might be helpful to stratify which patient would be at higher risk of developing complete ...

Arterial tortuosity syndrome (ATS) is a rare condition with autosomal recessive inheritance characterized by connective tissue abnormalities. The most specific clinical findings are cardiovascular anomalies including tortuosity, lengthening, aneurysm, and stenosis formation of major arteries. Also ventricular hypertrophy is frequently present. Other anomalies are skin hyperextensibility and cutis laxa, joint ...

Congenital aneurysm of the membranous portion of the ventricular septum in association with absence of atrioventricular valve 'offsetting' was diagnosed in two fetuses at 29 and 34 weeks. In the first case the fetus had a normal karyotype and no other structural heart defects, whereas in the second case there ...

We report a case in which aortopulmonary window (APW) in combination with pulmonary atresia was diagnosed correctly in a neonate by echocardiography. Prenatal echocardiography showed progression of tetralogy of Fallot to pulmonary atresia with retrograde pulmonary perfusion, concealing the concomitant APW in fetal life. Due to intractable heart failure, primary ...

BACKGROUND: Noonan syndrome is a rare disease, mainly presenting with malformations such as dysplasia and stenosis of the pulmonary valve, atrial septal defect and a typical pattern of hypertrophic cardiomyopathy. We report a case of Noonan syndrome with giant coronary aneurysms. CASE REPORT: A young woman with the phenotypic characteristics ...

AIMS: We tested whether procedural success of percutaneous septal ablation for hypertrophic obstructive cardiomyopathy is related to quantitative measurements of intraprocedural myocardial contrast echocardiography. METHODS AND RESULTS: In a study group of 34 patients, the mean area of the contrast depot was 8.5+/-2.5 cm2, its length along the left ventricular ...

Formation of a fully functional artery proceeds through a multistep process. Here we show that Notch3 is required to generate functional arteries in mice by regulating arterial differentiation and maturation of vascular smooth muscle cells (vSMC). In adult Notch3-/- mice distal arteries exhibit structural defects and arterial myogenic responses are ...

Congenital heart defects are the most common type of birth defect and contribute the most to infant mortality due to birth defects. This study examined the relationship between several demographic factors and selected congenital heart defects among the unique multiethnic population in Hawaii during 1986-99, using data from a population-based ...

Prenatal diagnosis of tetralogy of Fallot with pulmonary atresia (TOF/PA) was confirmed in a newborn with a birth weight of 2,095 g. Additionally, an aortopulmonary window (APW) type I was diagnosed on echocardiography. The operation was performed at the age of 4 weeks due to congestive heart failure. The APW ...

Vascular development depends on the highly coordinated actions of a variety of angiogenic regulators, most of which apparently act downstream of vascular endothelial growth factor (VEGF). One potential such regulator is delta-like 4 ligand (Dll4), a recently identified partner for the Notch receptors. We generated mice in which the Dll4 ...

Secundum atrial septal defects (ASDs) are routinely closed using transcatheter devices. In patients with left superior vena cava (LSVC) draining to the coronary sinus (CS), the device must not obstruct CS drainage. We report five cases of successful ASD device closure without obstructing flow from the LSVC or dilated CS.

Pulmonary vein isolation (PVI) is widely practiced as a means of potentially curing atrial fibrillation (AF). Transeptal puncture is performed for PVI procedures, often two such punctures are performed. We sought to examine the prevalence of medium- to long- term iatrogenic septal defects after such procedures. Thirty-one patients who were ...

BACKGROUND: Restrictive atrial septal defect (ASD) (including intact atrial septum [IAS]) has been reported to be a risk factor that negatively impacts survival in hypoplastic left heart syndrome (HLHS). Although lymphangiectasia and "arterialization" of the veins of the lung in HLHS with restrictive ASD have been reported, they cannot fully ...

BACKGROUND: Adjustment of pulmonary artery banding (PAB) may be a challenging procedure in complex congenital heart defects. Whatever the technique used, subsequent re-operations are frequently needed to control the pulmonary blood flow or pressures. OBJECTIVE: To report the efficacy of a new telemetric adjustable PAB (FloWatch-PAB) operated with the help ...

The aim of the present study was to determine if patients with both pulmonary arterial hypertension (PAH), due to pulmonary vascular obstructive disease, and congenital heart defects (CHD), have mutations in the gene encoding bone morphogenetic protein receptor (BMPR)-2. The BMPR2 gene was screened in two cohorts: 40 adults and ...

A congenital fistula between the right pulmonary artery and the left atrium is a rare condition that results in cyanosis. We report a successful catheter-based closure of such a fistula in a 12-year-old using an Amplatzer duct occluder. The patient also had an atrial septal defect that was closed with ...

After 2 years of medical management with a beta-adrenoreceptor blocking agent, a 30-month-old castrated male Golden Retriever with subaortic stenosis was treated surgically because of progression of its condition. In an attempt to achieve complete relief of the left ventricular outflow obstruction, a modified Konno procedure consisting of right ventriculotomy ...

Transcatheter closure of atrial septal defects has evolved significantly over the last 20 years. Transcatheter closure has replaced surgical closure for the treatment of most secundum atrial septal defectsat the current time. A major reason for this is the lower morbidity of transcatheter closure procedures. Several closure devices have come ...

Presenilin 1 (PS1) is the gene responsible for the development of early-onset familial Alzheimer's disease. PS1-deficient mice have been reported to show defects in neurogenesis, somitogenesis and angiogenesis. Here, we report cardiac anomaly in PS1-deficient mice: the mutant hearts exhibited ventricular septal defect, double outlet right ventricle, and stenosis in ...

INTRODUCTION AND BACKGROUND: About 1% of live-born children have congenital malformations of the heart. The aim of our study was to investigate the incidence of such defects in children born in Iceland during a period of 10 years, extending from 1990 through 1999. MATERIALS AND METHODS: Information about the patients ...

So-called 'absent pulmonary valve syndrome' is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe ...

Controversy over patients with complete atrioventricular septal defect (CAVSD) and coarctation of the aorta (CoA) continues: whether they should be treated with a primary total correction or a staged approach utilizing initial pulmonary artery banding during neonatal period. A 17-day-old neonate with Down's syndrome underwent definitive repair of CAVSD and ...

The authors report a case of double-orifice mitral valve (DOMV) which showed mitral stenosis and mild insufficiency. An associated anomaly was secundum atrial septal defect. DOMV is an unusual congenital heart defect. The occurrence of this anomaly with or without secundum atrial septal defect is very rare. More often it ...

Apart from closure of atrial septal defects, there is little information concerning the use of the CardioSEAL family of occluders in congenitally malformed hearts. We review here our initial experience using the CardioSEAL and STARFlex occluders in 12 patients aged 17.3 +/- 11.2 years, with a range from 4 to ...

Macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) is characterized by macrocephaly, cutis marmorata, capillary malformations, toe syndactily, joint laxity and pre-natal overgrowth. Cerebral abnormalities might also be seen. We reported cerebral magnetic resonance imaging (MRI) findings of a case with M-CMTC, who had giant atrial septal aneurysm and atrial septal defect. Cerebral ...

OBJECTIVE: This study was undertaken to identify potential anatomic and surgical factors creating left-sided lesions, namely recoarctation of the aorta and neoaortic regurgitation, after anatomic repair of transposition of the great arteries with ventricular septal defect and aortic coarctation. METHODS: From 1983 to September 2002, 109 survivors out of 120 ...

Re-circulation artefact developing secondary to vortex flow at the bulb of the internal carotid artery is very difficult to distinguish from true stenotic defect on two-dimensional Fourier transformed time-of-flight magnetic resonance angiography (2D-FT TOF MRA). The purpose of our study is to identify appropriate distinguishing features of re-circulation artefact. We ...

A 70-year-old man was admitted with sudden onset of dyspnea, near syncope and left hemiparesis. Clinical examination, blood gas analysis, ECG and the chest X-ray were consistent with pulmonary embolism. Transthoracic echocardiography (TTE) revealed right atrial free-floating thrombus, large atrial septal defect (ASD), right heart enlargement and pulmonary artery hypertension. ...

BACKGROUND: Stenoses of the neo-pulmonary artery (NPA) may complicate follow-up of the arterial switch operation (ASO). It is unknown whether the type of patch covering the coronary excision defects ("O"- or "U"-shaped) might influence this complication. METHODS: Echocardiographically and invasively measured NPA pressure-gradients were evaluated retrospectively in 95 children after ...

The authors described a rare case of spontaneous repositioning of an embolised Amplatzer occluder into the left atrium resulting in complete occlusion of a hemodynamically significant atrial septal defect, in the fossa ovalis, in a 70-year-old man. Only a slight central residual shunting was present, as was shown by transesophageal ...

Acute device thrombosis is a rare but important complication after transcatheter atrial septal defect closure. In this case a mobile thrombus was noted on the left side of an Amplatzer atrial septal occluder after device release in a 12 year old boy with an uncomplicated atrial septal defect. The thrombus ...

We present two cases of pulmonary atresia with ventricular septal defect who were not suitable for corrective surgery due to absent or hypoplastic native pulmonary arteries and were quite symptomatic following shunt surgery. We dilated and stented stenosed aortopulmonary collaterals as palliative procedure with improvement in oxygen saturation, and significant ...

Defects within the interatrial septum (IAS) can be a source of significant right-to-left shunting and hypoxemia, particularly after placement of a left ventricular assist device (LVAD). We report a case of LVAD placement in which an unrecognized IAS tear occurred intraoperatively, leading to profound arterial desaturation. Transesophageal echocardiography (TEE) was ...

The association of Leber's hereditary optic neuropathy (LHON) and left ventricular non-compaction in the absence of other neurologic or cardiac abnormalities has not been reported. In a 19-year-old man with deteriorating visual acuity, first in the right and two weeks later also in the left eye, LHON was diagnosed, based ...

Several clinical series have reported successful closure of perimembranous ventricular septal defects using trancatether devices. This review presents the morphological aspects of ventricular septal defects focusing on perimembranous septal defects. By definition, a part of the margin of perimembranous septal defects is the area of fibrous continuity abutting the central ...

A one-year-old female neutered beagle was presented with marked abdominal effusion. Echocardiography showed marked dilatation of the right cardiac chambers, an atrial septal defect and severe tricuspid insufficiency. Systolic pulmonary arterial pressure (sPAP), evaluated by continuous wave Doppler echocardiography, was very high (80 mmHg), with a right to left interatrial ...

We encountered a very rare lesion, which included a dissection between a prosthetic patch for repair of an incomplete atrio-ventricular septal defect (AVSD) and a pseudo-intima which had developed. This is the first report of a dissection between an atrial septum patch and pseudo-intima after mitral valve replacement (MVR) in ...

OBJECTIVES/HYPOTHESIS: The study aimed to detect the changes in adult rabbit nasal septal tissues after application of nasal pack or trans-septal suture as performed during septoplasty. STUDY DESIGN: Randomized double-blind animal study. METHODS: Thirty-three adult albino Vienna rabbits were assigned into the following groups: control, suture, and nasal pack. The ...

A 14-year-old female patient underwent surgical treatment of multiple atrial septal defects associated with unroofed coronary sinus and pulmonary valvar stenosis. One of the defects was that of the superior oval fossa and the other a large ellipsoidal defect positioned inferior to the inferior rim of the oval fossa. The ...

The success with occlusion devices for the closure of atrial septal defects and patent ductus arteriosus prompted the transcatheter closure of single and multiple muscular ventricular septal defects (VSD). The procedure for VSD was first attempted by Lock et al. in 1988 and devices originally designed for the closure of ...

We determined the accuracy of perfusion defect patterns in predicting the presence of proximal left anterior descending (LAD) artery stenosis in patients with angina pectoris. The development population consisted of 80 patients with single-vessel LAD stenosis and reversible LAD territory defects on thallium-201 tomography. The types of defect patterns associated ...

Congenital pericardial defect is a rare and little-known anomaly. Here we describe the unique clinical presentation of a 64-year-old man with partial defect of the left pericardium associated with ruptured acute type A aortic dissection manifesting massive left hemothorax. In this patient, the pericardial defect played the role of a ...

OBJECTIVE: Before the advent of antituberculous chemotherapy, thoracoplasty (TPL) was the definitive form of therapy for cavitary pulmonary tuberculosis. This study aimed to characterize the late functional sequelae of TPL, and to establish the degree of reversibility of any consequent airway obstruction. METHODOLOGY: Pulmonary function was studied in 21 long-term ...

OBJECTIVE: Abrupt increases in right ventricular afterload occur after cardiac transplantation and pulmonary artery banding, which can result in right ventricular hypertrophy and dilatation. Right ventricular dysfunction is also accompanied by beta-adrenergic receptor desensitization. We sought to determine whether selective right ventricular expression of a transgene encoding a beta-adrenergic receptor ...

Percutaneous closure of perimembranous ventricular septal defects (VSDs) has been feasible, safe, and effective with the new Amplatzer membranous septal occluder. We report further experience with this device with emphasis on morphological aspects of the VSDs and technical issues. Ten patients (median age and weight, 14 years and 34.5 kg, ...

Intracardiac echocardiography constitutes a new ultrasonographic window for the examination of the cardiovascular system, allowing a view of the heart and great vessels through a transvenous approach. Its use in interventional procedures, such as closure of secundum atrial septal defect and patent foramen ovale, transseptal puncture and radiofrequency ablation for ...