Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights.

BACKGROUND: About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. PATIENTS AND METHODS: A retrospective study of pediatric Wilms tumors treated at three sites (January 1999-June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. RESULTS: Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd-Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5-20 years), overall survival was 89% and event-free survival was 78%. CONCLUSIONS: Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect.

OBJECTIVES: To determine the early, intermediate and long-term outcomes of pulmonary atresia with ventricular septal defect (PA/VSD) Types I, II and III initially palliated by a right ventricle to pulmonary artery (RVPA) connection. METHODS: We performed a retrospective study from 2000 to 2014 that included 109 patients with PA/VSD who had undergone an RVPA connection (tetralogy of Fallot and PA/VSD Type IV excluded). The end-points of this strategy were adequate pulmonary artery tree post-palliation, second palliation, biventricular repair, right ventricular pressure post-biventricular repair and late reoperation. Mean follow-up was 5.4 years (1 day to 14-78 years). RESULTS: Early mortality after an RVPA connection was 2.7% (3 of 109). The interstage mortality rate was 6.6% (7 of 106). Eighty-four (77%) patients had a biventricular repair and 8 patients (7%) are awaiting repair. Overall survival was 90% at 1 year and 81% at 10 years. The RVPA connection allowed significant growth of the native pulmonary artery with a Nakata index of 101 mm2/m2 before the RVPA connection and 274 mm2/m2 after (P = 0.001). Twenty-nine reinterventions for restrictive pulmonary blood flow have been done (9 before 2 months and 20 after 2 months). Of the 84 patients who had a repair, 22 patients (26%) initially had a right ventricular pressure greater than 40 mmHg. Twenty-eight patients (33%) required late reoperation. CONCLUSIONS: Hospital deaths after the RVPA connection were low. The procedure allowed good growth of the native pulmonary artery. Biventricular repair was possible in a large number of cases. The late morbidity rate remains significant. Early reinterventions could be avoided by appropriate calibration. This technique appears to be suitable for any type of PA/VSD with central pulmonary arteries.

Dilatable pulmonary artery band: Safety and efficacy of balloon dilatation.

OBJECTIVES: Review early and midterm results of dilatable pulmonary artery band (PAB). BACKGROUND: PAB is performed in various conditions: as a transient stage before ventricular septal defect (VSD) closure, as a palliative procedure for multiple VSD or to prepare the left ventricle (LV) before switch or double switch operations in transposition of the great artery (TGA) or congenitally corrected transposition of great arteries (CCTGA). METHODS: All children with dilatation of PAB were reviewed. Reason for PAB and for cardiac catheterization, hemodynamics, and outcomes were analyzed. RESULTS: Between 2002 and 2014, 28 patients were identified. Reason for interventional dilatation of the PAB was supra systemic right ventricular (RV) pressure in four patients, aortic recoarctation in one patient, cyanosis in seven patients, RV failure in one patient, suprasystemic LV pressure in two CCTGA patients and spontaneous closure of VSD in other patients. All patients underwent successful balloon dilatation using high (n = 21) or low pressure balloons with a mean diameter of 12 mm. 23 Patients had no further interventions. Five patients had reintervention: one needed two dilatations of PAB and four had surgery (one double switch, one vicious pulmonary artery (PA) band removal, one in a context of severe RV dysfunction with tricuspid regurgitation, and one for VSD closure). One patient died of RV failure following surgery. CONCLUSION: Balloon dilatation of dilatable PAB carries good results with achievement of hemodynamic aim in most of the cases and definitive treatment in the majority of patients. Careful and sequential dilatation with shunt measurement is mandatory in patients with significantly residual VSD. © 2016 Wiley Periodicals, Inc.

Aortic root replacement in children: a word of caution about valve-sparing procedures.

OBJECTIVE: Evaluate the results of various surgical procedures used for aortic root replacement in children with aortic root aneurysm. METHODS: Between 1986 and 2007, 23 children (less than 16 years of age, mean age: 8.1+/-5.1 years) underwent elective aortic root replacement for aortic root aneurysm (with associated aortic insufficiency in 10 patients). All had connective tissue defect syndromes. Nine patients underwent composite valve graft repair using a mechanical valve. Fourteen children underwent valve-sparing aortic root replacement (remodeling procedure in 11, reimplantation procedure in 3). Mean follow-up (100% complete) was 7.3+/-5.5 years (range 6 months-21 years). RESULTS: There was one early death (4.3%)(after valve-sparing remodeling) and no late mortality. Following valve-sparing remodeling operation (10 patients, mean follow-up: 7.8+/-3.0 years), there were 9 reoperations in 6 patients and only 5 patients retained their native aortic valve. In patients who underwent valve-sparing reimplantation operation (three patients, mean follow-up: 2.1+/-0.3 years), one underwent reoperation for endocarditis. Fifteen patients had composite valve graft replacement either as a primary operation (nine cases) or at reoperation for valve-sparing failure (six cases); mean follow-up was 8.2+/-6.2 years; there was no thrombo-embolic or hemorrhagic event and one reoperation for patient-prosthesis mismatch. CONCLUSIONS: (1) composite valve graft aortic root replacement provides excellent long-term results in children with aortic root aneurysm due to connective tissue disorder. This remains the first choice procedure in patients with more than minimal aortic insufficiency, with distorted aortic leaflets or needing concomitant mitral valve replacement. (2) Valve-sparing remodeling surgery yields disappointing results and should probably be abandoned in the pediatric population. (3) Valve-sparing reimplantation repair may achieve superior outcome but needs further evaluation.

Myocardial revascularization in infants and children by means of coronary artery proximal patch arterioplasty or bypass grafting: a single-institution experience.

OBJECTIVE: We sought to evaluate midterm functional and anatomic results after coronary artery surgical arterioplasty or bypass grafting in infants and children. METHODS: Data concerning all consecutive patients operated on for myocardial revascularization in our institution between 1992 and 2004 were retrospectively analyzed. RESULTS: Twenty-five patients (mean age, 5.3 years) underwent surgical arterioplasty of a main coronary trunk, and this was for coronary obstruction after the arterial switch operation in 19 patients. Eight patients (mean age, 8.0 years) underwent a coronary bypass, and this was for postoperative coronary obstruction in all of them. One patient died 4 days after arterioplasty because of cardiogenic shock. One patient died suddenly 3.5 months after bypass from an unknown cause. All other patients were alive after a mean follow-up of 3.4 years after arterioplasty and 4.4 years after bypass. Among the 3 patients in whom the surgical enlargement of the left main trunk was extended to the left anterior descending coronary artery, 2 presented a restenosis of this artery and necessitated a coronary bypass 2.6 and 5.7 years, respectively, after arterioplasty. Among patients who had a postoperative angiogram, 17 (89%) of 19 after arterioplasty and 3 (50%) of 6 after bypass showed a good result. A internal thoracic artery graft was occluded, another one showed a complete string sign, and, finally, a patient presented with a tight stenosis of the bypass distal anastomosis. Eighteen (72%) patients after arterioplasty and 5 (63%) after bypass remained symptom free at last follow-up. CONCLUSIONS: Provided that the left main coronary artery bifurcation was not involved in the stenotic process, surgical arterioplasty of the main coronary trunks led to good functional and anatomic midterm results. On the other hand, variable indications and poorer preoperative cardiac conditions might have contributed to the disappointing results observed after coronary bypass.

Arterial aneurysms and tuberous sclerosis: a classic but little known association.

Tuberous sclerosis is a rare autosomal dominant syndrome in which aortic aneurysm is a classic but little known cause of sudden death. We report a 20-month-old boy with tuberous sclerosis and iliofemoral arterial aneurysms. This case highlights the importance of systematic screening for such vascular complications. Multislice CT or MRI is mandatory as a screening and diagnostic tool in this setting to complement US with Doppler examination.

The Ross procedure in infants and young children.

BACKGROUND: This study reviews our experience with the Ross procedure in infants and young children. METHODS: From September 1993 to September 2004, 52 children less than 15 years of age underwent a Ross procedure. The patients ranged in age from 4 days to 15 years old (median, 5 years). Fifteen patients (29%) were less than 2 years of age. The predominant indication for the Ross procedure was aortic stenosis. Sixteen patients underwent a Ross-Konno procedure for severe left ventricular outflow tract obstruction. Thirty-four patients had 48 previous interventions. Preoperatively, 6 patients showed severe left ventricular dysfunction, and 2 of the patients required ventilation and inotropic support. Concomitant procedures were performed in 8 patients. Three patients had a mitral valve replacement, 2 patients had a ventricular septal defect closure and an aortic arch reconstruction, 2 patients had aortic arch reconstructions, and 1 patient had resection of a coarctation and a ventricular septal defect closure. RESULTS: Patients were followed up for a median of 43 months (range, 1 to 130). Overall survival was 85% +/- 5% at 1 and 82% +/- 5% at 2, 5, and 10 years. Hospital mortality was 5 of 52 patients (9.6%). All deaths occurred in neonates or infants less than 2 months of age, who needed urgent surgery. Three patients died late of noncardiac causes. At last follow-up, all patients were classified in New York Heart Association functional class I or II. No patient had endocarditis of the autograft or the right ventricular outflow tract replacement. During the follow-up, no event of thrombembolism was observed. No patient required the insertion of a permanent pacemaker. Overall freedom from reoperation is 57% +/- 15% at 10 years. One patient required the replacement of the autograft at 6 months postoperatively. The development of mild aortic insufficiency was observed in 24 patients, and moderate aortic insufficiency in 1 patient during follow-up. Freedom from reoperation for the right ventricular outflow tract replacement is 60% +/- 15% at 10 years. CONCLUSIONS: The Ross procedure represents an attractive approach to aortic valve disease in young children. However, a high early mortality rate has to be considered when performing this procedure in neonates or infants who present in critical preoperative condition.

Orthotopic heart transplantation for congenital heart defects: anomalies of the systemic venous return.

Anomalies of the systemic venous return are frequently associated with other congenital heart defects. Some anomalies do not complicate really orthotopic heart transplantation (such as azygos continuation of the inferior vena cava). Other anomalies raise more difficulties; the most frequent one is persistent left superior vena cava draining into either the coronary sinus or the left atrium. Sometimes, the left superior vena cava can be ligated without untoward effect. In most cases, the left superior vena cava must be anastomosed to the right atrial compartment, preferably using extracardiac procedures. Most problems can be solved by harvesting extra lengths of donor superior vena cava and innominate vein. In rare patients, associated anomalies of the pulmonary venous return may require additional partitioning of the atria. The early risk of heart transplantation is probably not increased by the presence of such anomalies.

Orthotopic heart transplantation for congenital heart defects: situs inversus.

Although rare, visceroatrial situs inversus is not exceptional in patients with complex congenital heart defects. Achieving orthotopic heart transplantation using a graft harvested in a donor with situs solitus is surgically demanding. Technical problems can, however, be overcome by adequate harvesting of donor heart and by the use of innovative reimplantation techniques. These include: separate right and left pulmonary venous anastomoses, creation of an atrio-pericardial tunnel for reimplantation of the inferior vena cava, extracardiac reconstruction of the superior vena cava pathway using the donor innominate vein, direct aortic and pulmonary arterial reconstruction after adequate mobilization. There is evidence that the early risk of heart transplantation is not increased by the presence of recipient situs inversus.

Culture-negative pericarditis caused by Neisseria meningitidis serogroup C.

We describe a case of primary purulent culture-negative pericarditis caused by Neisseria meningitidis serogroup C occurring in an 8-month-old previously healthy boy, which was detected in pericardial fluid by broad-spectrum PCR amplification.

Histomorphometric analysis of pulmonary vessels in single ventricle for better selection of patients for the Fontan operation.

OBJECTIVE: In cases of single-ventricle physiology, the Fontan procedure often fails even when the usual selection criteria are strictly respected. We analyzed specimens from intraoperative open lung biopsies performed on 40 patients with single-ventricle physiology who were considered to be good candidates for the Fontan procedure. Histomorphometric study was performed to determine histologic factors predictive of failure of the Fontan procedure. METHODS: Histomorphometric studies were performed on samples from 40 patients aged 6 months to 23 years with single-ventricle physiology, either tricuspid atresia (n = 14) or univentricular heart (n = 26). The preoperative pulmonary arterial pressure was 18 mm Hg or less in 35 cases and greater than 18 mm Hg in 5 cases. Eighteen patients underwent total cavopulmonary connection, 16 patients underwent partial cavopulmonary connection, and 6 underwent a palliative procedure, as determined according to clinical and hemodynamic findings. RESULTS: Lung biopsy specimens from all 5 patients with pulmonary arterial pressure greater than 18 mm Hg appeared abnormal, whereas they appeared abnormal only 51% of the time in the low pulmonary arterial pressure group. The most frequent histologic abnormality observed was extension of smooth muscle cells in the wall of distal intra-acinar pulmonary arteries. Of the 18 patients who underwent the Fontan procedure, 9 had normal distal pulmonary arteries and good surgical results (except 1 with the Fontan circulation taken down for an anatomic reason). The remaining 9 had thick-walled distal intra-acinar pulmonary arteries with poor results of the Fontan procedure, and 6 died. The mean percentage wall thickness of small intra-acinar pulmonary arteries was significantly greater among the patients with bad results than among those with good results of the procedure (P <.01). CONCLUSIONS: Lung biopsy specimens were abnormal in 51% of patients with low pulmonary arterial pressure, there was no relationship between preoperative pulmonary arterial pressure and outcome, and extension of muscle in peripheral arteries was always present in cases of failure of the Fontan procedure. Histomorphometric study is therefore a useful adjunct to the usual selection criteria for surgical decision making in cases of single-ventricle physiology.