Percutaneous pulmonary valve implantation after endocarditis of Contegra® valved conduit: a case report.

This report describes the case of a 13-year-old boy with endocarditis of a Contegra® pulmonary valved conduit used for the correction of tetralogy of Fallot. He had severe pulmonary stenosis after endocarditis resolution. We performed percutaneous pulmonary valve implantation with resolution of the conduit dysfunction. The procedure was well tolerated by our patient and offered many advantages.

Bovine valved venous xenograft in pulmonary position: medium term evaluation of risk factors for dysfunction and failure after 156 implants.

AIM: This study evaluated risk factors for dysfunction and failure of the bovine Contegra valved conduit for right ventricle outflow tract reconstruction. METHODS: A total of 156 bovine jugular vein conduits were reviewed. Early and late mortality were evaluated. Seriate echocardiography was performed through the follow-up (mean 58±43.2 months, range 12 to 108 months), and risk factors and adverse events evaluation were analyzed by multivariate logistic regression. RESULTS: Hospital mortality was 7% (11/156). The only independent predictor of early mortality were cardiopulmonary bypass time >120 minutes and age <1 year. Postoperative mean RV to PA gradient was 10.4±9.1 mm Hg, increased to 18.7±14.5 mm Hg during the follow-up. A late gradient >30 mm Hg was assessed in 22 patients (15.1%),while 12 patients developed conduit valve regurgitation greater than 2+ without evidence of aneurismal formation. All patients with right ventricular (RV) to left ventricular (LV) pressure ratio >0.6 had conduit valve regurgitation. Acquired distal conduit stenosis was observed only in 1 patient. Thirteen patient underwent conduit replacement, diameter ≤14 mm was the only independent risk factor for reoperation. All explanted conduits were free from calcification and valve leaflets were intact and pliable. The Contegra graft itself was never cause of adverse event. CONCLUSION: The Contegra conduit provided excellent results and freedom from calcification in a medium-term follow-up. Younger age at operation was associated with higher need of replacements for conduit patient mismatch due to patient growth.

Cardiac defect with diaphragmatic hernia and left lung agenesis--heart disease and other anomalies.

This report describes a rare case of left pulmonary agenesis associated with congenital diaphragmatic hernia and congenital heart disease in a 2-year-old child with pulmonary hypertension. We performed direct radical correction of the congenital heart defect. The postoperative course was challenging but without major complications.

Ultrastructural pathology of pediatric myocardium in acute ischemia: bioptic study before and after treatment with cardioplegic solution.

Protecting the myocardium from the risk of acute ischemia during heart surgery is still an unsolved problem; the problem is even more open and more pressing in pediatric heart surgery. To meet this greater risk it is advisable to use a cardioplegic solution with a composition that is better suited to the particular morphofunctional conditions of the myocardium in the child, i.e., a solution offering greater protection. To this purpose the authors experimented with Celsior cardioplegic solution during heart surgery in children to evaluate the efficacy compared to the standard St. Thomas solution. In this comparative study 15 children were treated with Celsior cardioplegic solution and 15 others with St. Thomas cardioplegic solution. Each patient underwent 2 biopsies of the myocardium, the first before cardioplegic treatment and the second immediately after reperfusion. In both groups, focal lesions involving both the cardiomyocytes and the vascular-stromal structures were randomly found. The former had undergone a necrotic-regressive process with changes in the myofibrils and the mitochondria. The vascular-stromal structures showed changes in the permeability of the capillary endothelia, with interstitial edema. The results show the lesions to be similar in the 2 groups both on a quality and quantitative level.

Aortic valve homografts in the surgical treatment of complex cardiac malformations.

From April of 1968 to March of 1983, the surgical treatment of complex congenital cardiac malformations requiring an extracardiac conduit for their correction was performed with aortic valve homografts or aortic valved homograft conduits sterilized and preserved in our hospital. Our experience concerns 93 patients in whom a total of 103 aortic valve homografts were implanted. Ages of the patients ranged from 7 months to 36 years (mean 11.6 years). The aortic valve homografts were used from the right atrium to the pulmonary arteries or right ventricle (right atrium-dependent conduit), from the venous ventricle to the pulmonary arteries (ventricle-dependent conduit), or in the pulmonary orifice and in the superior and/or inferior venae cavae. There were 25 early and nine late deaths (36.5%), none of them related to the aortic valve homograft. The clinical follow-up of the 59 survivors (1 month to 15 years, mean 4.3 years) evidenced neither dysfunction of the aortic valve homograft nor thromboembolism or hemolysis; 93% of the patients are in New York Heart Association Class I or II. Control cardiac catheterization in 53 patients evidenced a pressure gradient in only 14 ventricle-dependent conduits. In seven patients with serial control catheterizations after 5 to 10 years, the pressure gradient had not increased.

Repeat syncopal attacks due to postsurgical right ventricular pseudoaneurysm.

Pseudoaneurysm of the right ventricular outflow tract is a rare lesion caused by disruption of the ventricular wall that allows the blood to leak into the surrounding space. It often complicates surgery involving right ventriculotomy and progressively increases in size, therefore causing airway compression, pulmonary perfusion asymmetry, thromboembolism, and rupture. We report on a patient who developed right ventricular pseudoaneurysm early after surgery for atrio-ventricular septal defect with tetralogy of Fallot and needed emergency surgical repair due to low cardiac output and repeat syncopal attacks.

Intrapericardial teratoma in the newborn.

Two cases of intrapericardial teratomas occurring in the newborn are reported. The first was a cystic, well differentiated tumour which was totally excised. The child was alive and well with no evidence of the disease 10 months later. The second was a predominantly solid teratoma which consisted of both mature and immature tissues. It was removed but recurred 2 months later with pleural and pericardial invasion. The patient underwent reoperation but death occurred 9 days after surgical treatment. The recurrence was histologically similar to the primary tumour. The second case is probably the first malignant intrapericardial teratoma described in a neonate. Its morphology and clinical behaviour suggest that, as in ovarian and sacrococcygeal teratomas, the presence of immature neuroepithelium carries a poor prognosis. In such cases radio- or chemotherapy should be performed.

Bacterial endocarditis of the aortic valve associated with diaphragmatic sub-aortic stenosis--account of two operated cases.

We report two cases of subvalvular aortic stenosis associated with a postendocarditic aortic valvular insufficiency. Although the aortic valvular disease had been diagnosed preoperatively, only the direct intraoperative observation and the histological examination of the removed valve revealed evidence of the endocarditis. Because of the seriously altered condition of the valvular tissue, treatment in both cases consisted of implantation of a mechanical prosthesis. Follow-up revealed no evidence of septic relapse.

Intermediate and late follow-up of the use of apico-aortic conduits in the surgical treatment of hypertrophic cardiomyopathy.

Between December 1977 and July 1983, four apico-aortic prosthetic valved conduits were implanted in four patients affected by severe hypertrophic cardiomyopathy. The disease was isolated in two cases, associated with critical stenosis of the right coronary artery in one case and with a subaortic tunnel plus aortic valve regurgitation in the final case. Three patients were male and one female; the ages ranged from 6 to 49 years. All patients survived surgery and were followed-up. At present, all are living and have no postoperative symptoms. The left ventricular-aortic gradient was relieved in all cases. Echocardiographic studies during the follow-up showed an enlargement of the left ventricular cavity with a satisfactory cardiac contraction. Three years following surgery the female patient gave birth to a normal male baby. Her pregnancy was normal and without complications. Seven years after surgery the first patient was successfully reoperated because of conduit thrombosis caused by degeneration of the biological porcine valve. In the follow-up, which ranges from 18 months to 7 years (mean 4.5 years) all patients are in Functional Class I. All preoperative symptoms have been relieved for all patients and there has been no incidence of late or sudden death.

Mechanical valve replacement under 12 years of age: 15 years of experience.

Despite improving surgical techniques, treatment of heart valve disease in children remains controversial. Growth of the child and adequate anticoagulation level are the main concerns when valve replacement is performed in the pediatric age. We reviewed the case histories of 29 children who underwent valve replacement with mechanical prosthesis from 1979 to 1994 in order to evaluate the performance of mechanical valves in this age group. Age ranged from two years to 12 years (mean 8.97 +/- 3.7 years). A total of 31 valves were implanted; 17 children had atrioventricular (Av) valve replacement (15 mitral, one common Av (heterotaxia), one tricuspid (systemic ventricle)), 11 children had aortic valve replacement (one redo), and one child had double mitral and aortic valves implanted. The etiology of valvular disease was congenital in 34.5%, degenerative in 17.2%, rheumatic in 24%, infective in 13.8%, and prosthetic dysfunction in 10.3%. Of the 29 patients, eight had undergone previous procedures and eight required simultaneous repair of associated lesions. There were three hospital deaths (10.3%). The mean follow up was 5.79 +/- 5.36 years. There were four late deaths (2.66%/patient-year) at a mean of 37 months from surgery. All operative survivors received oral anticoagulation with sodium warfarin. No thromboembolic event or bleeding occurred, no endocarditis developed in any patient; one reoperation was performed for patient/prosthesis mismatch. Mechanical valves offer excellent hemodynamic performances and a low rate of thromboembolism and/or bleeding in our experience, and are our first choice for heart valve replacement in children when reparative surgery is not feasible.

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.

Truncus arteriosus and double aortic arch associated with DiGeorge syndrome.

We report the 1st known case in which truncus arteriosus and double aortic arch have been associated with DiGeorge syndrome. The association of these 2 cardiovascular anomalies lends support, by itself, to speculation that truncus arteriosus and double aortic arch have a common embryonic pathogenesis; and the presence of these anomalies in a patient with DiGeorge syndrome strengthens the contention that the common causative factor is pathologic development of the neural crest cells.

Intermediate term evaluation of Starr-Edwards ball valves in the mitral position.

The Model 6120 ball valve prosthesis introduced in 1965 is still strongly supported as a mitral valve substitute in many centers around the world. A current reassessment of the performance of this prosthesis is therefore pertinent to current medical practice. In this institution since 1974, 227 Starr-Edwards caged ball valves have been implanted in the mitral position during isolated valve replacement. Two models of caged ball valves were used concurrently: the silastic ball valve in 108 patients (48%) and the composite strut "tract" valve in 119 (52%). Hospital mortality was 7%, and 8-year survival (standard error) was 74 (6%), with 100% follow-up, documenting 752 total patient-years. No late deaths were known to be valve related, and there were no cases of prosthetic thrombosis. The actuarial estimate of patients free from thromboembolism at 8 years was 89 (4%) with a linearized rate of 1.3% per year. At the most recent follow-up, 95% of the patients were in the New York Heart Association (NYHA) Classes I or II. These good results were partly due to an awareness at operation of ventricular outflow tract size requirements and to strict control of postoperative anticoagulation. We conclude that the Starr-Edwards ball valve is the mitral valve of choice in the young patient who is able to take anticoagulation drugs and has a left ventricular outflow tract of satisfactory size.

Technetium-99m methoxy isobutyl isonitrile simultaneous evaluation of ventricular function and myocardial perfusion in patients with congenital heart disease.

The authors evaluated the clinical applications of Tc-99m methoxy isobutyl isonitrile (MIBI) in the simultaneous assessment of ventricular function and myocardial perfusion in patients with congenital heart disease. Global ventricular function was assessed by first-pass ECG gated study. Myocardial perfusion was evaluated on images performed 1 hour after the injection of the tracer. Regional wall motion and systolic thickening were assessed by gated study acquired after the perfusion study. Two young patients were studied after a surgical Glenn procedure. The first patient, with a history of transposition of the great vessels and univentricular heart, had an ejection fraction of 44%. The left ventricle was dilated and the right ventricle was not appreciable. The septal and inferoapical regions showed reduced perfusion and reduced systolic thickening. The second patient, with a history of pulmonary atresia, septal defect and left ventricular hypoplasia, had a right ventricular ejection fraction of 37%. Regional wall motion, systolic thickening, and myocardial perfusion were normal. The right ventricle was hypertrophic and larger than the left ventricle. Thus, a single injection of Tc-99m MIBI allows noninvasive simultaneous assessment of global and regional ventricular function and myocardial perfusion in young patients with complicated congenital heart disease.

Hypertension in adult after operation of aortic coarctation.

AIM: The benefit of coarctation repair on the resolution of systolic hypertension in adults has been questioned. METHODS: Between March 1997 and July 2009, 65 consecutive adult patients (≥ 16 years) underwent repair of aortic coarctation. There were 40 men (65%) and 25 women (35%) with a mean age of 22.3 ± 4.8 years (range, 16 to 34 years). All patients had critical systolic blood hypertension (SBP ≥ 140 mmHg). SBP ranged from 140 to 205 mmHg, with a mean of 163.5 ± 17.6 mmHg. The mean diastolic BP was 95.1 ± 18.3 mmHg (range, 70 to 120 mmHg). Most patients (41/65, 74%) were on a regimen of at least one antihypertensive drug. RESULTS: The patients were followed up after coarctation repair for 2 to 144 months (mean, 68 ± 39 months). There was no death. No other major complications occurred. There have been no repeat interventions during follow-up. Four patients were lost to follow-up. Of the 61 patients with preoperative hypertension, 53 (87%) were normotensive (SBP <140 mmHg) at the most recent follow-up visit. The remaining eight patients showed substantial improvement versus the preoperative status. The mean SBP after operation was 122.5 ± 12.4 mmHg. Mean diastolic blood pressure was 79.5 ± 11.6 mmHg. Forty-one (67%) patients were taking no medication at the last follow-up. CONCLUSION: Surgical repair of coarctation of the aorta in adults can lead to regression of systolic hypertension and a decreased requirement for antihypertensive medication.