Francesco Seddio

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.

BACKGROUND Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

Shelhigh No-React porcine pulmonic valve conduit: a new alternative to the homograft☆

BACKGROUND The Shelhigh No-React pulmonic valve conduit is a new porcine conduit that is glutaraldehyde-treated and detoxified using a proprietary heparin process. In our institution it has been implanted in 25 patients. The aim of this present contribution is to evaluate the short-term follow-up after its implantation. METHODS From November 1997 to August 1999, 25 patients (mean age, 20.2 years; range, 0.6 to 28.3 years) were operated on using this conduit. Seventeen patients underwent a Ross procedure for aortic valve disease, with the conduits implanted in anatomic position; 6 patients underwent right ventricular outflow tract reconstruction; 2 patients underwent the Rastelli operation. The follow-up was complete. Preoperative and postoperative two-dimensional echocardiography data were collected. RESULTS There were two non-conduit-related deaths. Two conduits needed to be exchanged because of an increase in the gradient. Overall, all patients were improved in terms of New York Heart Association class. Comparison of preoperative and postoperative two-dimensional echocardiography gradient showed significant improvement. At the 30-month follow-up, no calcification was seen on the explanted conduits or on the two-dimensional echocardiography, although many of the patients are children. CONCLUSIONS The Shelhigh conduits seem to be an alternative to homograft especially in infants. These experiences are preliminary, and longer follow-up is required.

Is heart transplantation for complex congenital heart disease a good option? A 25-year single centre experience

OBJECTIVES Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors affecting mortality. METHODS Between 1985 and 2011, among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided into four age subgroups: <1 year (8 patients, Group I), 1-10 years (20 patients, Group II), 11-18 years (24 patients, Group III) and >18 years (33 patients, Group IV) and into two time periods: 1985-2000 (47 patients) and 2001-2011 (38 patients). Anatomical diagnoses were single-ventricle defect in 37 patients (44%) and two-ventricle defect in 48 patients (56%). Seventy-three patients (86%) had undergone one or more cardiac surgical procedures prior to HTx (mean 2.4 ± 0.9). Twenty-two of them were suffering from Fontan failure. Mean pulmonary artery pressure was 25.2 ± 14.2 mmHg. Mean transpulmonary gradient was 9.4 ± 6.9 mmHg. RESULTS Mean follow-up after HTx was 7.8 ± 6.8 years. Survival at 1 month was 37.7% in Group I, 85.8% in Group II, 96.8% in Group II and 98.4% in Group IV and was significantly worse in younger recipients. Overall 30-day mortality was 17.6%. Currently 56 patients (65.8%) are alive. Overall survival at 1, 5, 10 and 15 years is 83-, 73-, 67- and 58%, respectively. There were 14 late deaths. Univariate analysis found that risk factors for early and late death were those related to recipient illness, such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-losing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as an independent risk factor for mortality. Number of previous procedures did not influence survival. Previous Fontan procedure did not increase mortality. We documented the reversibility of PLE in survivors. CONCLUSIONS We demonstrated that heart transplantation for patients with CHD can be performed with the expectation of excellent results. Previous procedures, including the Fontan operation, do not reduce survival. Mortality is related to preoperative patient condition. We advocate early referral of complex CHD patients for transplant assessment and for inclusion in waiting lists before the detrimental effects of end-stage failure manifest themselves.

Levels of Troponin I and Cardiac Enzymes After Reinfusion of Shed Blood in Coronary Operations

BACKGROUND Reinfusion of shed blood after coronary artery bypass grafting might increase the levels of cardiac enzymes with consequent difficulties in the diagnosis of perioperative myocardial infarction. METHODS Thirty consecutive patients undergoing coronary artery bypass grafting who bled at least 400 mL within the first 4 hours after operation underwent reinfusion of shed blood. Thirty consecutive patients who were not autotransfused served as control. All patients underwent enzyme determination (total creatine kinase, MB fraction, lactate dehydrogenase, and troponin I) in the shed blood and in circulating blood preoperatively, at arrival in the intensive care unit, and 6, 24, and 48 hours after operation. RESULTS The shed blood contained significantly higher concentration of cardiac enzymes than the circulating blood at all time intervals (p = 0.0001). The levels of creatine kinase, its MB fraction, and lactate dehydrogenase in circulating blood were significantly elevated in patients receiving autotransfusion up to 24 hours after autotransfusion. The blood levels of troponin I were not significantly different between the two group of patients at all time points. The percent fraction of MB did not increase after autotransfusion. CONCLUSIONS The measurement of cardiac troponin I is a useful marker for the diagnosis of perioperative myocardial infarction in patients undergoing transfusion of shed blood after coronary operation.

Systolic anterior motion after mitral valve repair: myectomy as an alternative solution

by systolic anterior motion (SAM) of the mitral apparatus. 1 Correction of postrepair SAM is first medical: -blockers and calcium-channel blockers might be of some help in reducing left ventricular contractility. However, when a severe LVOT obstruction develops, a more aggressive approach is generally needed. In some cases obstruction of the LVOT disappears after removing the prosthetic ring; in other cases mitral valve replacement is necessary. When a hypertrophic septum is suspected to be a contributing factor leading to obstruction of the LVOT, a possible solution might be to perform a septal myectomy. Here we describe 2 cases of postrepair LVOT obstruction that were successfully treated by means of a transaortic septal myectomy in patients with no preoperative obstruction of the LVOT.

Intrapericardial Teratoma in a Newborn: A Case Report

Abstract lntrapericardial teratomas are unusual tumors that often cause respiratory distress and might be lethal in the newborn. The purpose of this article is to present the clinical and pathological findings in a 12‐day‐old male successfully treated for a big intrapericardial teratoma. Given that the diameter of this tumor is generally related to the age at the time of diagnosis, the rarity of our case is the presence of a huge intrapericardial teratoma in a newborn. The surgical resection was lifesaving.

Coronary Artery Involvement of Williams Syndrome in Infants and Surgical Revascularization Strategy

Williams syndrome (WS) is a genetic disorder due to deficiency of elastin gene expression. It is characterized by typical somatic abnormalities and a wide range of cardiovascular malformations. Coronary artery involvement is a frequent finding of the syndrome, particularly in those patients with severe supravalvular aortic stenosis. We present the case of an 11-month-old infant affected by WS who developed severe coronary artery disease 2 months after the surgical repair of supravalvular aortic stenosis. The clinical picture and successful surgical revascularization strategy is also described.