Jürgen Hörer

Unloading of right ventricle by bidirectional superior cavopulmonary anastomosis in hypoplastic left heart syndrome patients promotes remodeling of systemic right ventricle but does not improve tricuspid regurgitation

OBJECTIVE To evaluate the influence of volume unloading by bidirectional superior cavopulmonary anastomosis on the systemic right ventricle in patients with hypoplastic left heart syndrome. METHODS A total of 90 consecutive patients with hypoplastic left heart syndrome, who had survived the early postoperative period after bidirectional superior cavopulmonary anastomosis, were studied. Seven patients were excluded because of tricuspid valve surgery before or in association with bidirectional superior cavopulmonary anastomosis. The echocardiograms of the remaining 83 patients were reevaluated for tricuspid valve regurgitation and the size of the tricuspid annulus before bidirectional superior cavopulmonary anastomosis and at the last available follow-up examination before total cavopulmonary connection. RESULTS Echocardiograms were performed a median of 5 days before bidirectional superior cavopulmonary anastomosis. Tricuspid valve regurgitation was graded as 0 in 11 patients, I in 37 patients, II in 24 patients, and III in 11 patients. Follow-up echocardiograms were performed a median of 17 months after bidirectional superior cavopulmonary anastomosis. Postoperatively, tricuspid valve regurgitation was graded as 0 in 14 patients, I in 37 patients, II in 21 patients, III in 6 patients, and IV in 5 patients. Postoperatively, the mean Z value of the tricuspid annulus stayed the same in patients with significant tricuspid valve regurgitation (grade III or IV) after bidirectional superior cavopulmonary anastomosis but had decreased in the remaining patients. No significant change was seen in the level of tricuspid valve regurgitation after bidirectional superior cavopulmonary anastomosis compared with the preoperative data. CONCLUSIONS The relative size of the tricuspid annulus in patients with hypoplastic left heart syndrome decreases after bidirectional superior cavopulmonary anastomosis, most likely owing to volume unloading and promotion of the remodeling of the systemic right ventricle. However, this remodeling of the right ventricle does not improve the grade of tricuspid regurgitation.

Treatment of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome

OBJECTIVES To determine the incidence of right ventricle-to-pulmonary artery (RV-PA) conduit stenosis after the Norwood I operation in patients with hypoplastic left heart syndrome (HLHS), and to determine whether the treatment strategy of RV-PA conduit stenosis has an influence on interstage and overall survival. METHODS Ninety-six patients had a Norwood operation with RV-PA conduit between 2002 and 2011. Details of reoperations/interventions due to conduit obstruction prior to bidirectional superior cavopulmonary anastomosis (BSCPA) were collected. RESULTS Overall pre-BSCPA mortality was 17%, early mortality after Norwood, 6%. Early angiography was performed in 34 patients due to desaturation at a median of 8 days after the Norwood operation. Fifteen patients (16%) were diagnosed with RV-PA conduit stenosis that required treatment. The location of the conduit stenosis was significantly different in the patients with non-ringed (proximal) and the patients with ring-enforced conduit (distal), P = 0.004. In 6 patients, a surgical revision of the conduit was performed; 3 of them died prior to BSCPA. Another 6 patients had a stent implantation and 3 were treated with balloon dilatation followed by a BSCPA in the subsequent 2 weeks. All patients who were treated interventionally for RV-PA conduit obstruction had a successful BSCPA. Patients who received a surgical RV-PA conduit revision had a significantly higher interstage (P = 0.044) and overall mortality (P = 0.011) than those who received a stent or balloon dilatation of the stenosis followed by an early BSCPA. CONCLUSIONS RV-PA conduit obstruction after Norwood I procedure in patients with HLHS can be safely and effectively treated by stent implantation, balloon dilatation and early BSCPA. Surgical revision of the RV-PA conduit can be reserved for patients in whom an interventional approach fails, and an early BSCPA is not an option.

False aneurysm origination from the proximal anastomosis of a right ventricular to pulmonary artery shunt following staged repair of hypoplastic left heart syndrome

The Norwood I operation, including placement of a shunt from the right ventricle to the pulmonary arteries, has been adopted by many surgeons for neonates with hypoplastic left heart syndrome. A three-year-old male who had undergone the Norwood I operation, and the Glenn operation, presented with a cervical pulsating tumor prior to the operation for total cavopulmonary connection. At the Glenn operation, the right ventricle to the pulmonary artery shunt was closed with a clip proximally, and the distal part was resected. Following the Glenn operation, the child had had recurrent deep sternal infections caused by Serratia marcescens. Cardiac catheterization showed a false aneurysm from the proximal shunt anastomosis. The bleeding after resternotomy was managed by initiating cardiopulmonary bypass via the groin vessels. Cerebral air embolies were prevented by systemic application of potassium, to achieve cardioplegic arrest during chest opening. The shunt was removed and the defect was closed. After the shunt was confirmed to be free from infection, a total cavopulmonary connection was performed after three days postoperatively. The case illustrates the management of retrosternal aneurysms during resternotomy in children.

Combined epicardial and transvenous placement of an implantable cardioverter defibrillator (ICD) lead without a median sternotomy in an 8-year-old child.

An alternative to median sternotomy for epicardial placement of an implantable cardioverter defibrillator (ICD) lead in a child with hypertrophic cardiomyopathy is described. Implantation of an ICD lead via the tricuspid valve was avoided by the use of an epicardial pacing lead and a transvenous defibrillator lead placed in the vena brachiocephalica. The abdominal, subcostal pocket incision was used for an anterolateral minithoracotomy to implant the epicardial pacing lead.

Predonation of autologous blood reduces perioperative allogenic transfusion requirement in grown-up patients with congenital heart disease

BACKGROUND Adults with congenital heart diseases have a substantial risk for bleeding upon re-operations. Due to the detrimental effects of allogeneic blood transfusion, reduction of transfusion requirement is a major concern. To investigate the efficacy of autologous blood predonation (ABP), we focussed on a homogeneous subgroup of patients, with right ventricular outflow tract reconstruction. METHODS Prospectively collected data included 76 patients older than 16 years with repeated right ventricular outflow tract reconstruction from May 1995 to November 2006. In 27 patients, ABP was performed without any complication. RESULTS Primary diagnoses included Tetralogy of Fallot in 50 patients and others in 26 patients. All patients had at least one previous operation, 62% had more than one. All patients received a homograft conduit between the right ventricle and the pulmonary artery. Preoperative haemoglobin was 123+/-15 g l(-1) in patients with ABP and 134+/-22 g l(-1) in the remainder (p=0.037), but was not significantly different after cardiopulmonary bypass until discharge from the intensive care unit. Significantly more patients without ABP required transfusion of allogeneic packed red cells (PRCs) (26 of 49 patients (53%) vs 4 of 27 patients (15%), p=0.001) and allogeneic fresh frozen plasma (FFP) (30 of 49 patients (61%) vs 6 of 27 patients (22%), p=0.002) than patients with ABP. Of 27 patients, 23 (85%) and 25 (93%) with ABP received their predonated PRC and FFP, respectively. Logistic regression analysis identified no ABP (p=0.005, odds ratio (OR) 5.4, 95% confidence interval (CI) 1.7-17.7) and time on extracorporeal circulation >83 min (p=0.009, OR 5.0, 95% CI 1.5-16.8) to be predictive for allogeneic blood transfusion. CONCLUSION ABP can be safely performed in grown-up patients with congenital heart disease without complications. Patients without predonation of autologous blood exhibit a fivefold increased risk for requiring allogeneic blood transfusion.