1/395. Successful correction of double-outlet right ventricle with a ventricular D-l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect. The authors present the case of a fifteen-year old girl with double outlet right ventricle with ventricular d-loop and l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect, who was operated on successfully. This is the fourth case of double outlet right ventricle with l-position of the aorta that has been surgically corrected. The subaortic position of the interventricular defect favours the creation of the tunnel connecting the left ventricle with the aorta without obstructing the right ventricular outflow tract. The patient was doing well 11 months postoperatively. ( info) |
2/395. Right internal jugular vein approach as an alternative in balloon pulmonary valvuloplasty. Transjugular balloon pulmonary valvuloplasty was safely and successfully performed in five patients, two of whom had severe right heart failure. Crossing the stenotic pulmonary valve from the right internal jugular vein was consistently easy and quick. This approach should be considered whenever difficulties are experienced or anticipated in the femoral approach. ( info) |
3/395. Ebstein's anomaly associated with severe valvar pulmonary stenosis: successful palliation with balloon pulmonary valvuloplasty in an adult. We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention. ( info) |
4/395. phentolamine improves clinical outcome after balloon valvoplasty in neonates with severe pulmonary stenosis. Two neonates with severe pulmonary stenosis deteriorated immediately after successful balloon valvoplasty as a result of increased infundibular obstruction. They were treated with beta-adrenoceptor blockers and intravascular expansion with limited success. phentolamine was then given, resulting in dramatic improvement. The children could be weaned from the ventilator within hours of starting this new therapeutic modality. ( info) |
5/395. Unruptured congenital aneurysm of the sinus of valsalva presenting with pulmonary stenosis. Congenital aneurysm of the sinus of valsalva is a rare cardiovascular anomaly. It is usually silent until rupture occurs. The natural history of unruptured aneurysm of the sinus of valsalva is still not clear, and the therapeutic strategy is uncertain. Here we reported a case of unruptured aneurysm of the sinus of valsalva which was correctly diagnosed before invasive diagnostic procedures. A 30-year-old female noted mild palpitation and dyspnea for 1 month. Physically, a grade 3/6 systolic ejection murmur at upper left sternal border was detected. echocardiography revealed dilatation and irregular protrusion of the right sinus of valsalva encroaching on right ventricular outflow tract to cause obstruction. With these findings, unruptured aneurysm of the sinus of valsalva with pulmonary stenosis was diagnosed. cardiac catheterization and angiography confirmed the diagnosis. The aneurysm was repaired with a Dacron patch with good results. It is concluded that sinus of valsalva aneurysm can be diagnosed by echocardiography before its rupture so as to render a proper management for this potentially life-threatening anomaly. ( info) |
6/395. Pulmonary stenosis and severe biventricular dysfunction: improvement following percutaneous valvuloplasty. A 15-year-old boy with severe pulmonary stenosis associated with severe right and left ventricular systolic dysfunction is reported. After successful percutaneous pulmonary valvuloplasty, there was an initial and early improvement in right ventricular (RV) function, followed by a delayed and more gradual improvement in left ventricular (LV) function. At long-term follow up, both RV and LV systolic functions were nearly normalized. Several mechanisms may be implicated, including ventricular interdependence, geometric factors, altered compliance and intrinsic alteration in the LV muscle. A delayed, but sustained, improvement in LV systolic function following relief of RV pressure overload suggests that the latter mechanism must have played an important role in the genesis of the LV dysfunction. Pulmonary stenosis associated with severe biventricular dysfunction may be treated primarily by percutaneous pulmonary balloon valvuloplasty with near-total recovery of the ventricular function. ( info) |
7/395. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure. Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure. ( info) |
8/395. Pulmonary stenosis caused by extrinsic compression of an aortic pseudoaneurysm of a composite aortic graft. Pulmonic stenosis and stenosis of the right ventricular outflow tract related to extrinsic compression have been described in patients with tumors, in a patient with a pericardial cyst, and in patients with vascular abnormalities as an unruptured sinus of valsalva aneurysm, a giant coronary artery pseudoaneurysm and an aortic arch aneurysm. Composite graft replacement of the ascending aorta and aortic valve with reimplantation of the coronary arteries has some inherent complications. Our case report describes a patient with a pericomposite graft aneurysm presenting as a stenosis of the pulmonary artery, detected by Doppler echocardiography. ( info) |
9/395. Membranous subpulmonic stenosis associated with ventricular septal defect and aortic insufficiency. We present a case report of a patient with conal septal hypoplasia (supracristal) ventricular septal defect (VSD) complicated by aortic insufficiency (AI) and subpulmonic stenosis from a fibrous membrane. The development of AI with VSD is a well-recognized problem. However, the association of VSD, AI, and significant right ventricular outflow tract obstruction (RVOTO) is less common. Mechanisms of RVOTO include prolapse of an aortic valve cusp across the VSD, as well as infundibular hypertrophy or muscle bundles. Technical echocardiographic issues can make the diagnosis of VSD, AI, and RVOTO challenging. The presence of a discrete fibrous subpulmonary membrane is uncommon in this setting. ( info) |
10/395. Relief of an early pulmonary pathway obstruction with stenting after modified Fontan operation in an adult. The prognosis of tricuspid atresia, a rare congenital heart disease, has been changed by surgery. The criteria for Fontan operation have been well established in the literature and adult patients rarely fulfil these criteria; however, in very selected cases Fontan operation can be performed also in adults. A 33 year old woman with tricuspid atresia and previous palliation with classical right Glenn and with left modified Blalock-Taussing, underwent modified Fontan operation because increasing cyanosis and moderate exercise intolerance. Three weeks after operation the patient was readmitted with severe heart failure because of a tight obstruction at the anastomosis between right atrium and main-left pulmonary artery. The stenosis was treated with balloon and stenting achieving large pathway. Our experience confirms that after a modified Fontan, if cardiac failure occurs, an immediate full investigation have to be done. ( info) |