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.

A classical case of the Gasul phenomenon.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy - the classical Gasul phenomenon.

20 years of arterial switch operation for simple TGA.

BACKGROUND: Arterial switch operation became the golden treatment for simple transposition of the great arteries (sTGA). We describe our experience with the arterial switch operation regarding long-term outcome and the need for re-intervention. Nevertheless, supravalvular pulmonary stenosis (SPS) remains a concern in the long run. We assess the evolution of SPS over time and evaluate the effect of technical modifications on SPS during our experience. METHODS: We performed a retrospective study on 133 patients operated with ASO for TGA between October 1991 and November 2009. Last report method was used. We reviewed our pediatric cardiology and cardiac surgery database to examine the echocardiographic data and electrocardiograms. A mean follow-up of 9.2 years (+/- 5.83 SD) was reached. RESULTS: One (0.8%) patient deceased postoperatively due to cardiogenic shock. The overall actuarial freedom from reoperation (open and percutaneous) was 88.1%, 78.5% and 76.9% at 1, 5 and 10 years. SPS needed to be treated in 17 patients. Valve regurgitation at final investigation was maximal moderate in 5 patients for the aortic valve, 10 for pulmonary valve and 3 in tricuspid valve. CONCLUSIONS: ASO shows excellent long-term results in sTGA with a very low morbidity and mortality and is therefore the procedure of choice. Re-intervention rate is determined by SPS. Since the extensive mobilization of the pulmonary arteries and the creation of a longer neo-pulmonary root, reduction in SPS was seen with no re-interventions in the second half of the group. To obtain a final comparison with the atrial switch operation, a longer Follow-up is necessary.

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.

Primary infundibular stenosis and pedigree analysis in three Golden Retriever littermates.

Three eight-week-old Golden Retriever puppy littermates were evaluated because of left basilar systolic murmurs and were diagnosed with primary infundibular stenosis. Pedigree analysis in this line was also performed to identify a mode of inheritance. All dogs were asymptomatic at the time of diagnosis; two of the three had congenital lesions in addition to primary infundibular stenosis. Two additional affected dogs were identified in the line, and pedigree analysis suggested an autosomal recessive mode of inheritance. Another, unrelated golden retriever was also identified with isolated infundibular stenosis in the record database. Primary infundibular stenosis should be considered in the differential diagnoses for golden retriever dogs with a left basilar systolic murmur, and is often associated with complex congenital cardiac disease. Primary infundibular stenosis may worsen in severity with time, and in this line of dogs an autosomal recessive pattern of inheritance is likely.

Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development.

Pseudoaneurysm of the right ventricle outflow tract (RVOT) is a rare complication in pediatric cardiac surgery. We report a patient who developed a right ventricular pseudoaneurysm 8 months after RVOT enlargement using a pericardial patch for infundibular pulmonary stenosis. Our patient was born with severe pulmonary valvular stenosis and treated with percutaneous balloon valvotomy in the neonatal period. Six months later, she developed infundibular pulmonary stenosis, which required surgical resection of right ventricle infundibular trabeculations and bovine pericardial patch enlargement. The postoperative period was normal. She was readmitted to hospital 5 months later complaining of wheezing, coughing and shortness of breath. Echocardiography showed a huge aneurysmal dilatation of the outflow patch in connection with the right ventricular cavity. The patient underwent resection of the pseudoaneurysm and former patch, followed by interposition of a bovine jugular vein conduit between the RVOT and pulmonary bifurcation. The early postoperative period was uncomplicated. On echocardiography, no significant residual gradient was measured through the conduit and there was no insufficiency of the valve. RVOT reconstruction with patch enlargement, homograft or conduit implantation can be the origin of pseudoaneurysms. Although their incidence is rare, they are often asymptomatic before becoming quite large and causing compression symptoms as in our patient with respiratory complaints due to airway compression. It is important to follow up these patients closely, especially in the first year after surgery since most aneurysms develop within 6 months of surgery.

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.

Clinical and echocardiographic features of primary infundibular stenosis with intact ventricular septum in dogs.

BACKGROUND: Primary infundibular stenosis is a rare congenital defect in which the right ventricle is divided into a proximal "high-pressure" chamber and a distal "low-pressure" chamber. The condition can be misdiagnosed as ventricular septal defect or valvular pulmonic stenosis and the disease severity underestimated. The purpose of this study was to provide a detailed clinical and echocardiographic description of this anomaly in a series of dogs. HYPOTHESIS: Several anatomic forms of infundibular stenosis exist. High resolution two-dimensional echocardiography could differentiate 3 gross anatomic substrates. Knowledge of the anatomy of the obstructing lesion could influence options for corrective interventions. ANIMALS: Thirteen dogs examined at the Ontario Veterinary College teaching hospital from 1994 to 2005 with an ultrasound diagnosis of subpulmonic stenasis. METHODS: A retrospective review was made of case records from 1994 to 2005. RESULTS: Thirteen dogs were identified as having primary infundibular stenosis, with apparent increased prevalence in Golden Retrievers (8/13, 62%) and Siberian Huskies (3/13, 23%). Three types of infundibular lesions were identified by ultrasound in 11/13 dogs: a fibrous diaphragm (6), fibromuscular (4), and muscular obstruction (1). Two dogs with a fibrous diaphragm underwent direct surgical dilation without the use of cardiopulmonary bypass or inflow occlusion, resulting in substantial reduction of the severity of stenosis. CONCLUSION AND CLINICAL IMPORTANCE: Accurate determination of the severity of the stenosis and the anatomy of the obstructing lesion are important in devising a treatment strategy. Recognition of the fibrous diaphragm by echocardiography identifies a subset of dogs potentially amenable to surgical dilation without the need for cardiopulmonary bypass.

Surgical management of infants with isolated supravalvular pulmonary stenosis: case reports.

Pulmonary stenosis (PS) can be seen from the right ventricular outflow tract to the peripheral pulmonary arteries. Most frequently, the obstruction occurs at the level of the pulmonary valve; however, it occurs less frequently at the infindibular level within the trabecular component of the right ventricle or within the pulmonary arterial pathways. Lesions at any of these levels can occur as part of more congenital cardiac malformations such as tetralogy of Fallot, complete transposition of great arteries, or atrial septal defect. Isolated supravalvular pulmonary stenosis (iSPS) is less common than other types of PS. In this study, we present our experience with 4 patients who underwent cardiopulmonary bypass operation for iSPS. In one patient, the circular stenotic area was noted on the touch point of the pulmonary valve. Right ventricular pressures ranged from 70 to 90 mmHg, and the pulmonary artery mean pressures ranged from 14 to 17 mmHg. In all patients, the left ventricular and aortic systolic, diastolic, and mean pressures were moderately increased. Pulmonary artery stenosis was treated successfully using a pericardial or Dacron patch on cardiopulmonary bypass. Various techniques such as balloon dilation have been proposed to deal with this problem, but these may often be unsuccessful because of the elasticity and recoil of the pulmonary artery constrictive ring. Even though endovascular stenting and/or balloon angioplasty have been recently proposed as an initial treatment strategy, they may be associated with some severe complications including pulmonary artery thrombosis or stent migration. Our study, even though it consists of a limited number of cases, suggests that open heart surgery using an oval-shaped patch may be a used as the other main choice for the treatment of iSPS.

[Double chamber right ventricle. About 3 cases]. / Ventricule droit a double chambre. A propos de trois observations.

The double chambered right ventricle is a rare congenital abnormality. It creates an obstacle for the right ventricular ejection. The cardiac ultra sonography examination allows the diagnosis in most majority of cases. The angiography is performed in difficult cases. We report 3 cases of infundibular stenosis with normal interventricular septum. The diagnosis was suspected for the 3 cases by the constellation of clinical findings pointing to systolic murmur over the precordium, and the electrocardiography showing right ventricular hypertrophy. It is confirmed by the echocardiogram and the hemodynamic exploration with a trans stenotic gradient evaluated at 72,80 and 80mmHg. The 3 patients underwent surgery and had all good post operative follow up. These 3 observations allow us to recall the epidemiological, embryological, clinical and echocardiographic particularities of pure infundibular stenosis and to determin the adequate treatment and the prognosis.

Echocardiographic differentiation of infundibular from valvular pulmonary stenosis.

Echocardiographic tracings of the pulmonary valve were examined in 24 normal subjects, 16 patients with valvular pulmonary stenosis and 3 patients with infundibular pulmonary stenosis. In normal subjects, atrial contraction produced a slight posterior opening motion of the pulmonary valve leaflet (a wave). This presystolic opening motion (a wave) varied with respiration, and maximal a wave depth recorded during quiet inspiration (Amax) averaged 3.7 plus or minus 1.2 (standard error of the mean) mm (range 2 to 7 mm). In the 10 cases with moderate or severe valvular pulmonary stenosis, increased force of right atrial contraction and elevated right ventricular end-diastolic pressure resulted in an increased posterior or opening motion of the pulmonary valve leaflet, and Amax averaged 9.6 plus or minus 2.0 mm (range 8 to 13 mm, P less than 0.001 versus normal). When both anterior and posterior leaflets were recorded, presystolic opening or doming of the valve was observed. In six cases of mild valvular pulmonary stenosis, Amax averaged 4 plus or minus 2.5 mm (not significant). In patients with infundibular pulmonary stenosis, marked chaotic systolic fluttering of the valve leaflet, which lies in the turbulent stream of blood distal to the obstruction, was recorded. This finding was never seen with valvular pulmonary stenosis. In two cases of mild infundibular pulmonary stenosis, the amplitude of presystolic opening motion was within the normal range of 3 and 7 mm. In one case of severe infundibular pulmonary stenosis, no presystolic opening motion was recorded, thus suggesting that the small pressure changes produced by atrial systole failed to reach the valve leaflets. Echocardiography, therefore, should be of use in differentiating valvular from infundibular pulmonary stenosis.

False diagnosis of subpulmonary obstruction by echocardiography in d-transposition of the great arteries.

Twenty-one consecutive children (aged 3 to 14 months) with d-transposition of the great arteries were evaluated with standard M mode echocardiography at the time of hospital admission for routine preoperative cardiac catheterization. At catheterization, 6 of the 21 were found to have a systolic pressure gradient between the body of the left ventricle and the main pulmonary artery. Echocardiographic systolic anterior mitral valve motion was noted in nine patients, including five who did not have a left ventricular to pulmonary arterial systolic pressure gradient. Diastolic approximation of the anterior mitral leaflet to the septum was noted in 14 (9 without a gradient), pulmonary valve flutter was also present in 14 (9 without a gradient) and there was premature systolic pulmonary valve closure in 6 (3 without a gradient). Echographic left heart dimensions were not different in patients with a left ventricular outflow pressure gradient than in those without, but patients wit systolic anterior mitral valve motion and diastolic approximation of the mitral leaflet to the septum did have smaller left ventricular and pulmonary root dimensions than did those without these echocardiographic findings. Echocardiographic findings that have been described as suggesting left ventricular outflow obstruction in patients with d-transposition of the great arteries appear to be more related to size and configuration of the left ventricle than to the presence or absence of obstruction.

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.

Diagnosis and follow-up of congenital heart disease in children with the use of two-dimensional Doppler echocardiography.

Two-dimensional echocardiography (2D) represents a major advance in non-invasive diagnosis of congenital heart disease (CHD) in children. Nevertheless it has diagnostic limitations in nearly all kinds of heart lesions. These can be overcome for the most part by integration of a pulsed Doppler system. This may be called two-dimensional Doppler echocardiography (2DD). Hereby blood flow information is added to the 2D image. Some common types of CHD including ventricular and atrial septal defects, persistent ductus arteriosus, pulmonic stenosis and coarctation are described with their typical 2DD findings. Non-invasive follow up of children with CHD and early recognition of typical complications can be achieved reliably using 2DD. Future prospects consist in a more quantitative diagnostic application of 2DD.

Membranous subvalvular pulmonary stenosis in complete transposition of the great arteries: usefulness of serial exercise testing.

A 22-year-old male who had Mustard repair for complete transposition presented with progressive exercise intolerance, documented by a gradual decrease in exercise capacity on cycle ergometer. Cardiac catheterization and angiography demonstrated subvalvular pulmonary stenosis. After surgical relief of the obstruction, exercise capacity returned to previous level.

Isolated subpulmonic fibrous ring, mirror-image dextrocardia and situs solitus in a young lady unreported and a near miss.

Congenital diseases causing obstruction of the right ventricular outflow tract (RVOT) are common, but the isolated subpulmonary membrane/ring is extremely rare and can be difficult to diagnose precisely, especially in adults. We report a case of surgically resected isolated subpulmonic fibrous ring in a lady with mirror-image dextrocardia and abdominal situs solitus that was misdiagnosed by echocardiography as a subaortic membrane.