Giant right atrium and subvalvular pulmonary stenosis: A case report of an interesting combination.

A 20-year-old Congolese woman presented with presyncope, dyspnea, and anasarca. Past medical history was unremarkable. Echocardiography revealed a rare combination of giant right atrium (RA), a dilated and hypertrophied right ventricle, subvalvular pulmonary stenosis (subPS), severe tricuspid regurgitation (TR), pericardial effusion and what appeared to be a spontaneously closed ventricular septal defect (VSD). Cardiac Magnetic Resonance and Cardiac Computed Tomography confirmed the findings excluding the presence of intra-cardiac and extra-cardiac shunt and other associated congenital anomalies. The patient underwent subPS resection, right atrioplasty, and tricuspid annuloplasty. Multimodality approach facilitated the detection of the abnormalities and provided clarity when determining the optimal surgical strategy.

Severe subvalvular pulmonic stenosis due to a ball-shaped accessory tissue in an asymptomatic woman with congenitally corrected transposition of great arteries and mesocardia: A rare case report.

Congenitally corrected transposition of great arteries (CCTGA) is a rare anomaly described by atrioventricular and ventriculoarterial discordance. On the other hand, mesocardia that is extremely rare includes two relatively well-defined apexes defined by each ventricle with the major axis of the heart lies in the midline. We describe a rare case of an asymptomatic 20-year-old woman with mesocardia, CCTGA, and severe subvalvular pulmonic stenosis due to a ball-shaped accessory tissue in left ventricular outflow tract.

Ebstein's anomaly with subpulmonary obstruction--a rare association.

Ebstein's anomaly comprises a wide spectrum of congenital cardiac abnormalities involving the tricuspid valve (TV) apparatus and the architecture of the morphological right ventricle (RV). We report a case of Ebstein's anomaly in a 3-year-old boy having a unique association with severe subpulmonic obstruction caused by tethered and dysplastic anterior tricuspid leaflet (ATL), and a large ventricular septal defect. In view of poorly functioning RV, the baby was planned for a bidirectional Glenn shunt with subsequent Fontan completion.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

Transthoracic echocardiography does not reliably predict involvement of the aortic valve in patients with a discrete subaortic shelf.

BACKGROUND: A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve. METHODS: A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings. RESULTS: A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement - 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively - than found at surgery - 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker. CONCLUSION: Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.

Prosthetic aortic valve endocarditis complicated with annular abscess, sub-aortic obstruction and valve dehiscence.

We present a 76-year-old woman with infective endocarditis of a prosthetic aortic valve. The course of her illness started with an ischaemic stroke and she was admitted with prolonged fever and an episode of loss of consciousness. Echocardiography revealed acute aortic regurgitation and dehiscence of the prosthetic valve with excessive "rocking motion", aortic abscesses and left ventricular outflow obstruction caused by a semilunar shelf of tissue probably due to endocarditis vegetations. She underwent an urgent surgical procedure that confirmed the echocardiographic findings. Our case report reinforces the value of early diagnosis in the presence of a high clinical suspicion of prosthetic valve endocarditis. An extended workup, including transoesophageal echocardiography, in such a patient with a mechanical valve is mandatory.

Pulmonary infundibular stenosis and ventricular septum defect: usefulness of cardiac CT.

A 35-year-old man with a history of syncope and echocardiographic diagnosis of right ventricular outflow tract obstruction and ventricular septum defect was submitted to complete evaluation with an Aquilion Toshiba 64-multislice CT in order to confirm diagnosis and to complete information prior to surgery.

Isolated pulmonic valve endocarditis in an adult patient with ventricular septal defect and infundibular pulmonary stenosis.

Ventricular septal defect associated with infundibular pulmonary stenosis is a relatively uncommon congenital cardiac defect. We report the first case of a patient with perimembranous small ventricular septal defect and infundibular stenosis suffered from pulmonary valve endocarditis and septic pulmonary embolism.

[Endovascular balloon valvuloplasty of pulmonary artery valvular stenosis].

Summarized experience of balloon valvuloplasty in isolated stenosis of pulmonary artery valve is analyzed. This procedure was performed in Russian Research Center of Surgery in 53 patients from 1980 to 2006. The technique of balloon valvuloplasty is described; short- and long-term results are analyzed. Complications and unsatisfactory results of this surgery are outlined.

A case report of Noonan's syndrome with pulmonary valvar stenosis and coronary aneurysms.

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 of Noonan's syndrome presented with severe pulmonary stenosis and giant coronary aneurysms. Cross sectional echocardiography showed valvar and subvalvar pulmonary stenosis. The valve itself was thickened and dysplastic, a characteristic that is typical of Noonan's syndrome. In addition to the usual abnormalities of the pulmonary valve and the ventricular myocardium, the patient showed a wide spectrum of previously unreported coronary aneurysms. CONCLUSIONS: These additional findings support the hypothesis that a vasculitic process has been superimposed on the connective tissue defect associated with Noonan's syndrome. Furthermore, since the pathogenesis of the condition remains unclear, this case stresses the need to look carefully for abnormalities co-expressed in Noonan's syndrome.

Acute effects of DDD pacing in patients with pulmonary infundibular stenosis.

We evaluated acute effects of DDD pacing (right atrium sensed and left ventricle paced) in 3 patients with pulmonary infundibular stenosis and found a decrease in dynamic right ventricular outflow gradient in all of them. It appears that acute temporary DDD pacing may decrease the dynamic obstruction of the right ventricular outflow tract in these patients, probably because of asynchronous contraction of the right ventricle induced by pacing from the left ventricular apex, with contraction of infundibular portion being delayed.

Subpulmonary and subaortic ridges in doubly committed subarterial ventricular septal defect: an echocardiographic study.

BACKGROUND: Certain conditions, including discrete subaortic stenosis and right ventricular outflow tract obstruction are well-known associations with ventricular septal defect. However, the association of discrete type subpulmonary and subaortic stenosis with ventricular septal defect has not been described to date. PATIENTS: In this report we present our experience in nine patients with doubly committed subarterial ventricular septal defect in whom associated discrete subpulmonary and subaortic stenosis were diagnosed by two-dimensional echocardiography. The mean age of the patients was 5.7 years, and eight of them were male. Two patients had additional hypertrophied anomalous muscle bundles with pressure gradients across the right ventricular outflow tract. Aortic valve prolapse was detected in two patients with one of them having mild aortic regurgitation. INTERVENTION: Cardiac catheterization was performed in four patients, and three underwent surgery. Doubly committed subarterial ventricular septal defect was closed, and subpulmonary and subaortic ridges were resected in each patient. Histological findings were similar for both ridges, which were composed of thin, short and irregularly arranged elastic fibres covered by endothelial cells. RESULTS: The finding of fibrous ridge on both sides of the doubly committed ventricular septal defect, where the turbulence is likely to be maximum, and the histopathological similarities of subpulmonary and subaortic ridges, may suggest a common mechanism for the development of fibrous ridges. CONCLUSION: Serial echocardiographic assessment of patients with doubly committed ventricular septal defect is recommended.