Role of sildenafil in acute posttransplant right ventricular dysfunction: successful experience in 13 consecutive patients.

BACKGROUND: Superimposed acute right ventricular dysfunction in the setting of preexisting pulmonary hypertension is a nearly fatal complication after heart transplantation. The optimal treatment modality remains a matter of debate. Recently, sildenafil citrate, a nonselective pulmonary vasodilator, has gained popularity in the treatment of pulmonary hypertension in transplant candidates. METHODS: Herein we have presented a series of 13 patients in whom sildenafil was used to treat right ventricular dysfunction and pulmonary hypertension as detected by transesophageal echocardiography and Swan-Ganz right heart catheterization after heart transplant. Their characteristics were mean age 49+/-11.4 years; 38.4% with previous cardiac procedures, 30.8% status I, basal pulmonary vascular resistance index 10.4+/-4.6 WoodU, mean transpulmonary gradient 18.7+/-5.4 mmHg. In addition to conventional inodilator support, we administered 1 to 3 mg per kilogram of sildenafil. Complete hemodynamic measurements were obtained before and after the institution of the therapy and at 1-month follow-up. RESULTS: Within the first 72 hours, acute right ventricular dysfunction resolved in all cases without untoward side effects or significant systemic impact. Sildenafil significantly decreased the transpulmonary gradient and pulmonary vascular resistance index relative to baseline values; 5.6+/-1.82 versus 10.4+/-4.6 WU, (P< .05), 13.5+/-3.4 mm Hg versus 18.7+/-5.4 mm Hg (P< .05), respectively. Improved indices of right ventricular function were observed on echocardiographic monitoring. After 1 month, sildenafil treatment was discontinued. CONCLUSION: Management of acute right ventricular dysfunction in heart transplant recipients with pulmonary hypertension using sildenafil proved safe and effective.

Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery.

Double outlet right ventricle associated with atrioventricular concordance, pulmonary stenosis and situs solitus of the atria is a subset of double outlet right ventricle related through the surgical treatment. From 1974 to 1985, 14 patients, 5 males, 9 females (mean age 8.9 years, range 13 months-22 years) were operated upon. All patients had infundibular stenosis and normal or large pulmonary arteries. The apex of the heart was to the right in 2 patients, the right and left ventricles were superior and inferior in 2 patients and 1 patient had both anomalies. The ventricular septal defect was subaortic in 11 patients (aorto-mitral discontinuity in 5) and non-committed in 3 patients. Three patients had 2 ventricular septal defects. The aorta was anterior in 3 patients and to the right of the pulmonary artery in 11 patients. All patients, through a transventricular and transatrial approach, had a reconstructive surgery. In 3 patients, an aortic homograft valved conduit was used. One patient had the ventricular septal defect enlarged. There was one early death (7.1%) from high residual right ventricle pressure and no late death. One patient had a transient atrioventricular block. One patient was reoperated upon for a residual ventricular septal defect. All survivors had a good clinical result. Re-evaluation in 8 patients confirmed excellent haemodynamics: the right ventricle to pulmonary artery pressure gradient decreased from 80 mm Hg (range 60-95) preoperatively to 24 mm Hg (range 3-32) postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Right pulmonary artery development after modified Blalock-Taussig shunt (MBTS) in infants with pulmonary atresia, VSD and confluent pulmonary arteries.

The ideal palliation for infants with pulmonary atresia, ventricular septal defect and confluent pulmonary arteries should promote normal development of the pulmonary artery branches. In 26 patients who survived a modified Blalock-Taussig shunt (MBTS) in the first year of life, the right pulmonary artery was measured before and after operation by two dimensional (2D) echocardiography. In each patient its size was compared to the normal value for the same body surface area. The patients were divided according to this ratio and according to the real size (mm). Although increased in all cases but two, the size of the right pulmonary artery remained remarkably less than normal in patients with an initially small right pulmonary artery. In view of these results, early palliative enlargement of the right ventricular outflow tract is probably advisable in infants with diminutive pulmonary arteries who do not show adequate pulmonary branch development after MBTS.

[Endoscopic retrograde catheterization of Vater's ampulla in the diagnosis of bilio-pancreatic diseases]. / I1 cateterismo retrogrado endoscopico della papilla di Vater nella diagnostica delle affezioni bilio-pancreatiche

The technique, indications and contraindications for endoscopic retrograde cholangiopancreatography are discussed. Some of the most demonstrative cholangiographic and pancreatographic pictures are described and the reasons that have led to a marked reduction in complications immediately after the examination are analysed. Therapeutical aids for limiting any slight disturbances connected with the examination are also presented. Finally, some morphological data useful for differentiating between inflammatory and neoplastic lesions in the juxta- and intrapancreatic biliary excretory way and the pancreas itself are considered.

The effect of aortic coarctation surgical repair on QTc and JTc dispersion in severe aortic coarctation newborns: a short-term follow-up study.

Sudden death is a possible occurrence for newborns younger than 1 year with severe aortic coarctation (CoA) before surgical correction. In our previous study, we showed a significant increase of QTc-D and JTc-D in newborns with isolated severe aortic coarctation, electrocardiographic parameters that clinical and experimental studies have suggested could reflect the physiological variability of regional and ventricular repolarization and could provide a substrate for life-threatening ventricular arrhythmias. The aim of the current study was to evaluate the effect of surgical repair of CoA on QTc-d, JTc-d in severe aortic coarctation newborns with no associated congenital cardiac malformations. The study included 30 newborns (18M; 70+/-12 h old) affected by severe congenital aortic coarctation, without associated cardiac malformations. All newborns underwent to classic extended end-to-end repair. Echocardiographic and electrocardiographic measurements were performed in each patient 24 h before and 24 h after the interventional procedure and at the end of the follow-up period, 1 month after the surgical correction. All patients at baseline, 24 h and one month after CoA surgical repair did not significantly differ in terms of heart rate, weight, height, and echocardiographic parameters. There were no statistically significant differences in QTc-D (111.7+/-47.4 vs 111.9+/-63.8 ms vs 108.5+/-55.4 ms; P=0.4) and JTc-D (98.1+/-41.3 vs 111.4+/-47.5 vs 105.1+/-33.4 ms; P=0.3) before, 24 h and 1 month after CoA surgical correction. In conclusions, our study did not show a statistically significant decrease in QTc-D and JTc-D, suggesting the hypothesis that the acute left ventricular afterload reduction, related to successful CoA surgical correction, may not reduce the ventricular electrical instability in the short-term follow-up.

Stenting of the arterial duct in newborns with duct-dependent pulmonary circulation.

OBJECTIVE: To evaluate the feasibility and results of stenting of the arterial duct in newborns with duct-dependent pulmonary circulation using low-profile, high-flexibility premounted coronary stents. DESIGN: Prospective interventional and clinical follow-up study. SETTING: Tertiary referral centre. PATIENT POPULATION: Between April 2003 and December 2006, 26 neonates (mean (SD) age 15.2 (19.9) days, mean (SD) weight 3.3 (0.8) kg) underwent attempts at stenting of the arterial duct. MAIN OUTCOME MEASURES: Procedural success and complication rates. Early and mid-term follow-up results. RESULTS: The procedure was successfully completed in 24/26 (92.3%) cases. Minor complications occurred in 2/26 (7.7%) cases. No mortality occurred. After stenting, the ductal diameter increased from 1.2 (1.0) mm to 3.1 (0.4) mm (p<0.001) and the percutaneous O(2) saturation increased from 70 (14)% to 86 (10)% (p<0.001), respectively. Over a mid-term follow-up, 2/24 patients (8.3%) needed a systemic-to-pulmonary artery shunt because of inadequate ductal flow and 4/24 patients (16.7%) underwent stent redilatation after 6.0 (4.4) months, but before corrective surgery. Cardiac catheterisation before corrective surgery in 9 patients showed an increase of the Nakata index from 112 (49) mm/mm(2) to 226 (108) mm/mm(2) (p<0.001), without any left-to-right imbalance of the pulmonary artery size. In the subset of 11 patients who improved without needing an additional source of pulmonary blood supply, the stented arterial duct closed uneventfully in 45.5% of cases after 4.0 (2.2) months. CONCLUSIONS: Stenting of the arterial duct is a feasible, safe and effective palliation in newborns with duct-dependent pulmonary circulation, supporting the spontaneous improvement process or promoting significant and balanced pulmonary artery growth for subsequent corrective surgery.

[Late traumatic rupture of the chordae tendineae]. / Rottura traumatica tardiva di corde tendinee.

A rare case of traumatic rupture of chordae tendineae with tricuspid regurgitation is described. The rupture occurred later trauma, with an unusual mechanism, in fact it has been produced by the strangling of valvular apparatus. This strangling occurred because heart's displacement in left thorax trough a pleural-pericardial window caused by trauma.

Critical left ventricular outflow tract obstruction due to accessory mitral valve tissue.

Left ventricular outflow tract (LVOT) obstruction due to anomalous tissue tag arising from the mitral valve is a rare congenital cardiac anomaly. It generally becomes symptomatic during the first decade of life as exercise intolerance, chest pain, or syncope at effort. To date, only a few cases of critical systemic obstruction due to isolated mitral valve anomaly in neonates have been reported. We report the case of a neonate who was a few hours old and was referred in severe clinical condition due to critical left ventricular outflow obstruction resulting from an anomalous tissue tag of mitral valve origin.

Pre-and postoperative Tc 99m scanning in monitoring pulmonary perfusion in the tetralogy of Fallot.

Angiocardiopneumographic scanning with Tc 99m was used in pulmonary perfusion and intracardiac shunt before and after radical corrective surgery of the tetralogy of Fallot. The study was carried out on 25 patients with the tetralogy of Fallot undergoing radical treatment with infundibular patch. Preoperative scanning has shown increased thickening of right ventricle and contemporary injection of pulmonary artery and aorta, the latter with a much higher radioactivity; the obstruction to the right flow was not clearly defined with respect to morphology and function, pulmonary perfusion has shown an extremely poor radioactivity and prolonged duration. Scanning following surgical treatment showed normal right and left phase and absence of residual shunts. Captation of marker in the lungs was always complete. Such data illustrate the many advantages gained by the use of non invasive diagnostic procedures in cardiology and "the first pass technique" in the evaluation of results of surgical treatment.