Rupture of the papillary muscle of the tricuspid valve - echocardiographic diagnosis of a rare anomaly leading to critical tricuspid valve regurgitation in the newborn.

We present the case of a male full-term neonate who presented at 4 h of age with severe cyanosis unresponsive to mechanical ventilation and oxygen supplementation. The diagnosis of rupture of the papillary muscle of the tricuspid valve was made by echocardiography. Preoperative stabilization was achieved by adding nitric oxide and extracorporeal membrane oxygenation therapy could be avoided. Corrective surgery was successfully performed on the 7th day of life. Follow-up examination at 2 months of age revealed an excellent postoperative result. Perinatal rupture of the papillary muscle and/or chordae tendineae of the tricuspid valve is exceedingly rare and usually lethal when untreated or diagnosed late. Therefore, timely diagnosis is mandatory for adequate preoperative management and subsequent surgical treatment.

Double discordance in the hypoplastic left heart.

A neonate was transferred for an intracardiac mass. Initially, the mass was mistaken for a hypoplastic right ventricle, a thrombus, or a tumor. Only a thorough segmental and sequential analysis showed double discordance with a hypoplastic left ventricle. No such entity has yet been described.

Catheter interventional treatment of Sano shunt obstruction in patients following modified Norwood palliation for hypoplastic left heart syndrome.

UNLABELLED: Shunts placed between the right ventricle and the pulmonary arteries, called Sano shunts, recently modified Norwood surgery for hypoplastic left heart syndrome. Patients with Sano shunts tend to be more stable thus reducing the interstage mortality of this still challenging complex cardiac anomaly. However, Sano shunt stenosis may develop and is a life threatening complication. We report on our experience in patients with Sano shunt obstruction. PATIENTS: Eight infants presenting with decreasing transcutaneous oxygen saturations (43-63%, median 58%) following modified Norwood procedures were shown to have relevant Sano shunt stenosis. None was suited for early stage two surgery (cavopulmonary Glenn anastomosis). Catheterization was performed at the age of 21 to 112 (median 85) days. Weight was 3.9 to 6.0 (median 4.8) kg. TECHNIQUE: Femoral 5F venous access. Long sheaths were not used. The shunt was entered with a 4F right Judkins catheter and a selective angiography was performed. The stenosis was localized proximal in 5, distal in 1 and proximal and distal in 2 patients. Ten coronary stents were implanted. RESULTS: There were no procedure related complications. Oxygen saturation increased immediately to 75-86% (median 80%) and remained above 70% during follow-up in all. Seven patients had successful stage two surgery 61-288 (median 134) days after stent implantation, one is awaiting this. CONCLUSIONS: Sano shunt obstruction can be treated safely and effectively by stent implantation. Early in-stent restenosis does not seem to be a problem.

Ross and Ross-Konno procedure in children and adolescents: mid-term results.

OBJECTIVES: The aim of the study was to analyze mid-term results of aortic root replacement with pulmonary autograft in children and adolescents in two centers. METHODS: From December 1997 through August 2003, a total of 66 patients underwent the Ross procedure in two centers. Indication for Ross procedure was predominantly aortic stenosis in 24 patients and predominantly aortic regurgitation (AR) in 22 patients. Twenty patients with severe left ventricular outflow tract obstruction (LVOTO) underwent Ross-Konno procedure. No patient had a geometric mismatch of more than 5 mm in favor of the aortic annulus. RESULTS: There was no early death. One patient died 3 months after surgery due to bacterial endocarditis. Survival on median follow-up period of 2.4 years was 98.5%. Neo-aortic regurgitation was none in 29 (44%) patients, trivial in 35 (53%) patients and mild in 2 (3%) patients. One patient (1.5%) needed aortic valve replacement because of autograft failure. Actuarial freedom from more than trivial neo-aortic regurgitation, or aortic valve replacement was 95% at 5 years follow-up. There was no patient either with recurrent LVOTO or significant aortic root dilatation. Freedom from redo was 93% at 5 years of follow-up. There had been a significant reduction (P = 0.001) and normalization in the left ventricle diastolic diameter index and left ventricle mass index, respectively, within 3-12 months after operation. Sixty-three percent of all operated patients are without medication; no one is on anticoagulation therapy. CONCLUSIONS: Our 7 years experience with the Ross and Ross-Konno operation has shown excellent mid-term results, with mortality rate approaching zero in both simple and complex left heart lesions, even in the neonates and infants. It is a procedure of choice in children with severe anomaly of the aortic valve and/or left ventricular outflow tract obstruction. The main concern is dilatation of the neo-aortic root leading to progression of AR, especially in the settings of geometric mismatch of aortic and pulmonary roots and bicuspid, regurgitant aortic valve. The risk of autograft failure in these specific subsets of patients remains to be determined.

Long term results of percutaneous balloon valvoplasty of congenital aortic stenosis: independent predictors of outcome.

OBJECTIVE: To evaluate long term results and independent predictors of outcome of aortic valvoplasty. DESIGN: Retrospective follow up study. Independent predictors of outcome identified by multiple logistic regression. SETTING: Tertiary referral centre. PATIENTS: 269 consecutive patients treated at the median age of 8 months (0-23 years): 80 (30%) under 4 weeks, 59 (22%) between 4 weeks and 1 year, and 130 (48%) over 1 year. The follow up period was up to 14.8 years (median 5.3, in survivors 6.4 years). INTERVENTIONS: Percutaneous balloon valvoplasty with mean (SD) balloon to annulus ratio 0.97 (0.08). MAIN OUTCOME MEASURES: Restenosis > or = 70 mm Hg, grade 3 aortic insufficiency, cusps disruption, surgery, death, and valvoplasty failure (significant restenosis or insufficiency or surgery or death). RESULTS: The mortality rate was 10.4% (n = 28), the restenosis rate was 16.7% (n = 45), significant insufficiency developed in 22.3% (n = 60), surgery was needed in 20.1% (n = 54), and "valvoplasty failure" occurred in 41.6% (n = 112) patients. Mean (SEM) survival probability 14.4 years after the procedure was 0.89 (0.02) and mean (SEM) probability of surgery-free survival was 0.50 (0.08). The independent predictors were as follows. For restenosis: small aortic annulus; for cusp disruption: large aortic annulus; for insufficiency: bicuspid aortic valve; for need for surgery: bicuspid aortic valve; for death: small aortic annulus, low left ventricular shortening fraction, and low sequential number of the valvoplasty; and for valvoplasty failure: small aortic annulus, bicuspid aortic valve, and high grade of mitral insufficiency. CONCLUSION: Independent predictors of unfavourable outcome are small aortic annulus, bicuspid aortic valve, poor function of left ventricle or mitral valve, and limited operator experience.