Double-orifice mitral valve repair.

We present the case of a 6-year-old patient with double-orifice mitral valve and severe regurgitation, which was successfully repaired. We demonstrate that mitral valve repair may be attempted even in complex anatomy such as the double-orifice mitral valve, where it can offer excellent results.

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.

Acquired infundibular pulmonary stenosis associated with a congenital membranous ventricular septal defect (Gasul phenomenon) in a dog and discussion regarding causes of infundibular stenosis.

An aclinical Havanese dog was diagnosed with a membranous restrictive ventricular septal defect. The patient was represented later in their natural history due to the development of syncope. At that time the patient was diagnosed with acquired pulmonary infundibular stenosis. Balloon dilation of the stenosis was performed successfully twice over the patient's lifetime. The patient died suddenly approximately 14 months after the second balloon dilation. A discussion regarding primary infundibular pulmonary stenosis versus causes of acquired infundibular pulmonary stenosis including anomalous muscle bundles (double chamber right ventricle), tetralogy of Fallot, and infundibular stenosis is presented.

Surgical correction of congenital heart disease at 76 years of age.

The management of adult congenital heart disease is challenging and poses specific problems. We report a patient with ventricular septal defect and pulmonary stenosis who underwent successful repair and coronary artery bypass grafting at the age of 76 years.

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.

Clinical manifestations of dynamic left ventricular outflow tract stenosis in infants with d-transposition of the great arteries with intact ventricular septum.

Four infants with d-transposition of the great arteries and intact ventricular septum who manifested early clinical symptoms and deterioration due to dynamic left ventricular outflow stenosis are presented. All four had an anatomically adequate atrial septal defect, made at the initial balloon atrial septostomy, that was later confirmed intraoperatively. Two infants continued to have a low arterial oxygen saturation level because of inadequate interatrial mixing, and one of these had severe persistent cyanosis and was treated with the Mustard operation at age 4 days. The other two infants subsequently presented with hypercyanotic spells at age 3 months. All four infants had features of dynamic left ventricular outflow stenosis on hemodynamic, angiocardiographic and echocardiographic studies. The left ventricular outflow pressure gradient was shown to increase after administration of isoproterenol in one infant, and relief of a cyanotic spell with reduction of left ventricular systolic pressure was achieved in another after intravenous administration of propranolol. The Mustard operation relieved symptoms in all infants. The effect of left ventricular outflow tract stenosis on the mechanisms responsible for interatrial mixing in d-transposition of the great arteries with intact ventricular septum is discussed.

One-stage repair of interrupted aortic arch, ventricular septal defect, and subaortic obstruction in the neonate: a novel approach.

BACKGROUND: One-stage repair of interrupted aortic arch, ventricular septal defect, and severe subaortic stenosis represents a surgical challenge. Techniques that use extracardiac conduits to bypass the subaortic area or involve transaortic or transatrial resection of the conal septum have shown limitations and have failed to reduce the high mortality rate associated with subaortic obstruction. METHODS AND RESULTS: A new operative approach was used in nine neonates (2.1 to 3.9 kg) who underwent one-stage repair of interrupted aortic arch (type B, eight patients; type C, one patient), ventricular septal defect, and severe subaortic stenosis. All patients had severe subaortic stenosis according to preoperative echocardiography (mean ratio of subaortic to descending aortic diameter, 0.63 +/- 0.08). With a transpulmonary (seven patients) or transatrial (two patients) approach and without resection of the conal septum, the ventricular septal patch was placed on the left side of the septum to deflect the conal septum anteriorly and away from the subaortic area. There were no early or late deaths. Median intensive care unit and hospital stays were 17 days (6 to 47 days) and 21 days (10 to 55 days), respectively. On follow-up echocardiography (1 to 29 months, median 12 months), no patients had significant residual subaortic obstruction and one patient had mild residual arch obstruction (20 mm Hg). Growth of the subaortic region was demonstrated in all patients (mean ratio of subaortic to descending aortic diameter, 1.20 +/- 0.10; < 0.001). CONCLUSIONS: Relief of severe subaortic stenosis during one-stage neonatal repair of aortic arch interruption and ventricular septal defect can be accomplished successfully without resection of the conal septum.

Situs inversus totalis associated with subaortic and subpulmonic stenosis.

The unusual occurrence of total situs inversus and idiopathic hypertrophic subaortic stenosis with the demonstration of right and left heart dynamic obstruction in one patient is presented. The fact that the patient was known to have a rare abnormality (total situs inversus) and the presenting symptom being angina pectoris, may have obscured the diagnosis of IHSS and emphasized the value of comprehensive evaluation of patients. The documentation of right ventricular dynamic obstruction, in addition to obstruction of the left side of the heart, was facilitated by the use of simultaneous pressure recordings in the pulmonary artery and in the body of the right ventricle. The angina-like pain was secondary to left ventricular outflow obstruction, rather than to coronary disease.

Allograft root on closed pulmonary valve for subaortic obstruction in double-inlet left ventricle with transposition of the great arteries.

BACKGROUND: Until recently closure of the pulmonary valve during staged Fontan-type palliation in the setting of double-inlet left ventricle with an unrestrictive or adequately enlarged ventricular septal defect and transposition of the great arteries with the aorta on a left-sided outflow chamber was regarded as an appropriate part of surgical treatment. Lately, however, an increased incidence of subsequent subaortic obstruction has been described in this regard. METHODS: Allograft root placement on the previously closed pulmonary orifice in combination with a modified Damus-Kaye-Stansel procedure is described to create an unobstructed outflow from the main ventricle to the systemic circulation. This procedure was done in 3 patients. One root placement was combined with the construction of the bidirectional superior cavopulmonary connection, one was done as an intermediate step before completion of the cavopulmonary connection, and one was combined with completion of total cavopulmonary connection. RESULTS: Immediate relief of the subaortic obstruction was achieved in all 3 patients. Ventricular hypertrophy, echocardiographically assessed by diastolic posterior wall thickness, regressed to normal in all 3 within 6 to 12 months. CONCLUSIONS: Allograft root placement on the reopened pulmonary orifice in double-inlet left ventricle with a ventricular septal defect and transposition of the great arteries appears technically feasible and functionally adequate on short-term follow-up. This procedure should result in regression of ventricular hypertrophy to allow eligibility for a Fontan-type palliation again. To what extent possible failure of the allograft increases the risk of an adverse outcome of this palliation may be a matter of concern.

Surgical correction of unusual double-outlet right ventricle.

This paper presents the case history of an 8-year-old girl who had total situs inversus and double-outlet right ventricle with pulmonary stenosis and severe tricuspid insufficiency in the presence of dextrocardia with ventricular discordance. A successful repair was performed using the Rastelli technique in conjunction with replacement of the tricuspid valve with a Bjork-Shiley prosthesis. The postoperative course was uneventful, and follow-up catheterization revealed a good operative result. However, the patient died suddenly during an emotionally upsetting period about two months after the operation. Postmortem examination revealed only signs of moderately severe cardiac decompensation. Some anatomical and embryological comments are made.

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.

Surgical repair of pulmonary stenosis with intact ventricular septum in a 68-year-old woman.

Patients with mild pulmonary stenosis after infancy rarely have symptoms or develop increasing obstruction. We experienced a 68-year-old woman with severe pulmonary valvar and infundibular stenosis (peak to peak pressure gradient = 80 mmHg). She had been pointed out heart disease at the age of six. Endocarditis at the age of 17 might induce calcification of valve and affect the progression of pulmonary stenosis, and moreover, which might gradually develop severe subvalvar obstruction and poststenotic aneurysm of pulmonary trunk. She refused operative intervention because of mild clinical symptoms (NYHA class II), but we recommended surgical repair due to repeated transient ischemic attacks, which were suspected paradoxical embolism through persistent foramen ovale. She underwent pulmonary valvotomy and infundibular resection and is doing well.

Sites of right ventricular outflow tract obstruction in tetralogy of Fallot--a retrospective study.

Fifty one angiographically proved cases of tetralogy of Fallot (TOF) in the age group of 5-50 years were analysed retrospectively for the level(s) of right ventricular outflow tract (RVOT) obstruction. Mean age was 18.1 +/- 11 yrs. Subvalvular stenosis was found to be the commonest site of RVOT obstruction in 49 (96.1%) patients. Evidence of valvular pulmonary stenosis was seen quite commonly (42/51, 83.9% cases), mostly in association with obstruction at other sites. Supravalvular stenosis was also seen in 17 (33.3%) cases. Higher incidence of pulmonary valvular involvement in patients with TOF, in higher average age of patient population may represent valvular involvement to be an acquired phenomenon.

Giant pulmonary artery aneurysm with right ventricular outflow tract obstruction.

Aneurysm of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines regarding its optimal treatment. We present a case of a huge saccular aneurysm of the main pulmonary artery which was associated with infundibular and valvular pulmonary stenosis. It was repaired using a pericardial patch with concomitant pulmonary valvotomy and infundibular resection. Postoperative recovery was uneventful and the patient is doing well. Follow-up echocardiogram revealed good repair.

[Obstructions to pulmonary ejection and restrictive interventricular septal defects]. / Obstacles à l'éjection pulmonaire et communications interventriculaires restrictives.

The association of a restrictive ventricular septal defect with a pulmonary tract obstruction is rare and may cause diagnostic and therapeutic problems. The authors report their experience of such an association in 10 children. 3 had a tetralogy of Fallot (1 death), 4 a double-outlet right ventricle (2 deaths), and 3 a coarctation of the aorta with ventricular septal defect and pulmonary artery banding (no death). Prognosis depends on the type of malformation. It has improved with years because of sooner recognition of these malformations and better surgical technics and post-operative care.

Bourneville's tuberous sclerosis associated with double outlet right ventricle and infundibular pulmonary stenosis.

The cardiac manifestation usually associated with tuberous sclerosis is rhabdomyoma of the heart. We report a rare association with cyanotic congenital heart disease in the form of double outlet right ventricle with infundibular pulmonary stenosis.