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.

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.

Conal anatomy in 119 patients with d-loop transposition of the great arteries and ventricular septal defect: an echocardiographic and pathologic study.

OBJECTIVES: We sought to study the range of conal morphology in transposition of the great arteries with ventricular septal defect and their embryologic and surgical implications. BACKGROUND: Conal anatomy in transposition of the great arteries and ventricular septal defect is variable and might affect surgical repair. METHODS: Conal anatomy was explored using two-dimensional echocardiography in 119 patients with transposition of the great arteries and a large ventricular septal defect who presented between 1984 and 1991. The influence of conal anatomy on surgical technique was determined by review of the operative reports. Specimens of transposition of the great arteries with unusual conal anatomy were selected from the Cardiac Registry for comparison with the echocardiograms. RESULTS: One hundred five patients (88.2%) had subaortic conus only with no subpulmonary conus (Group 1). Subarterial conus was present bilaterally in eight patients (6.7%) (Group 2). Four patients (3.4%) had only subpulmonary conus with no (or minimal) subaortic conus (Group 3). Among these four patients, the aorta was posterior to the pulmonary artery in one patient, side by side relative to the pulmonary artery in two patients and slightly anterior in the fourth patient. Subarterial conus was absent bilaterally in two patients (1.7%) (Group 4); the aorta was slightly posterior in one and side by side with the pulmonary artery in the other. CONCLUSIONS: This variability of conal anatomy in transposition of the great arteries with ventricular septal defect implies four mechanisms by which transposition can occur. The conal anatomy appeared to affect surgical repair in Groups 1 and 2 insofar as it influenced ventricular outflow tract obstruction. In Groups 3 and 4, an arterial switch operation was performed in four of the six patients. The posterior location of the aorta obviated the need for the Lecompte maneuver in two of these four patients. In the remaining two cases in Groups 3 and 4, the condition was repaired by directing the left ventricular outflow across the ventricular septal defect to the aorta using a patch, with or without placement of a conduit from the right ventricle to the pulmonary artery.

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.

Collateral vessels between the coronary and bronchial arteries in patients with cyanotic congenital heart disease.

Selective coronary arteriography (using the Sones technique) was performed in 67 patients with congenital heart disease aged 1 to 33 years. Five of the 23 patients with cyanotic congenital heart disease had collateral vessels between the coronary and bronchial arteries; none of the 44 patients with noncyanotic congenital heart disease had such vessels. Each of the five patients with collateral vessels had severe obstruction of the right ventricular outflow tract or the pulmonary valve plus a ventricular septal defect and a right to left shunt. It appears that such collateral vessels from the coronary arteries provide an increment to pulmonary blood flow in patients with cyanotic congenital heart disease and diminished pulmonary flow.

Right ventricular volume in congenital heart disease.

With use of the Frank lead system, still loop and timed vectorcardiograms were recorded in more than 5,000 patients subjected to complete right and left heart catheterization and selective coronary cineangiography. Data so obtained demonstrated clinical superiority of the vectorcardiogram over the standard 12 lead scalar electrocardiogram. Specific advantages of the vectorcardiogram include (1) recognition of undetected atrial and ventricular hypertrophy, (2) greater sensitivity in identification of myocardial infarction, and (3) superior capability for diagnosis of multiple infarctions in the presence of fascicular and bundle branch blocks. The timed biplane vectorcardiogram is as useful as any number of simultaneously recorded electrocardiographic leads for the analysis of complex arrhythmias and beat to beat changes in intraventricular conduction. Since the validity and usefulness of this technique have been established, it should become part of the routine noninvasive evaluation of patients with cardiovascular disorders.

Subpulmonic stenosis as a result of noncalcific constrictive pericarditis.

The clinical, electrocardiographic, and radiologic features in a patient with a pericardial band which produced an unusual form of infundibular pulmonary stenosis are presented. The findings are unique in that the band was not calcified and, therefore, not visualized roentgenographically and developed without a previous history of pericarditis or pericardial surgery. The diagnostic value of cineangiography is stressed and illustrated.

The transmitral approach to left ventricular outflow tract obstruction.

Left ventricular outflow tract obstruction is a particularly vexing problem in patients with complete transposition (atrioventricular concordance and ventriculoarterial discordance) and intact ventricular septum. Previous techniques, such as resecting the obstructive lesion through the pulmonary valve or bypassing the obstruction, have not given entirely satisfactory results. An alternative approach of resection through the left atrium and the retracted mitral valve has recently been proposed. We have used this new technique to achieve excellent relief of this condition in three patients with different types of obstruction.

Double mitral valve orifice. Two-dimensional and Doppler echocardiographic diagnosis.

The purpose of this study was to demonstrate the usefulness of two-dimensional and Doppler echocardiography for diagnosing double mitral valve orifices (DMVO) in addition to identifying associated pathologies. We report five cases, three male and two female with an age ranging from 4 to 44 years old (mean age: 17 years), with the diagnosis of DMVO according to the following characteristics: using two-dimensional echocardiography on the short parasternal axis, both orifices were observed; apical in which the "seagull sign" was identified in both chambers, in addition to identifying the flows of each orifice by pulsed and codified color Doppler obtaining the corresponding gradients. With respect to the associated pathologies, all patients presented some type of malformation, such as subaortic ring, patent ductus arteriosus, coarctation of the aorta, bicuspid aorta and pulmonary stenosis. Using the color Doppler echocardiography allows an adequate anatomical and functional definition of DMVO.