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.

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.

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.

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.

Repair of pulmonary artery sling by reimplantation without cardiopulmonary bypass.

A 20-month-old girl successfully underwent repair of pulmonary artery sling through a median sternotomy by division of the left pulmonary artery and its reimplantation into the main pulmonary artery without cardiopulmonary bypass or tracheal reconstruction. The patient is doing well on 18 months follow-up with unobstructed pulmonary blood flow and dramatic reduction of tracheal stenosis. Simple repair of pulmonary artery sling is feasible with good results in selected cases without tracheomalacia.

Left ventricular outflow tract obstruction in the transposition of great arteries defined by transthoracic three-dimensional echocardiography.

We assessed the feasibility of transthoracic three-dimensional reconstruction of the pulmonary valve and subpulmonary left ventricular outflow tract in two patients with transposition of great arteries, ventricular septal defect, and obstruction to the left ventricular outflow tract. Three-dimensional reconstruction of the pulmonary valve could be displayed as "en face" through a three-dimensional generated "pulmotomy view," allowing an overview of the pulmonary aspect of the valve from a surgeon's perspective. In similar fashion, reconstruction of the subpulmonary outflow tract could be displayed along its longitudinal axis as seen through a left ventriculotomy. Unique views could be obtained equivalent to surgical or autopsy dissections, allowing more complete understanding of the morphology and severity of left-sided obstructive lesions.

[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.

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.

Left ventricular mechanics after closure of ventricular septal defect: influence of size of the defect and age at surgical repair.

To evaluate the influence of the size of the defect and the age of surgical repair on left ventricular mechanics, including geometry, shape, diastolic and systolic function as well as myocardial contractility, we used cross-sectional echo-Doppler to study 20 patients (12 males, 8 females) who had undergone successful surgical closure of a ventricular septal defect. The patients were divided in two groups, corrected early and late, on the basis of the degree of left-to-right shunting (ratio of pulmonary to systemic output of greater or less than 2.5/1) and the age at the surgical repair (older or younger than 2 years of age). The group undergoing early correction included 11 patients, mean age 7.1+/-1.8 years (range 4.2-11.8 years), having surgery at mean age of 1.3+/-0.6 years for a large ventricular septal defect (mean ratio of pulmonary to systemic output of 3.1/1; range 3.4-2.7/1) with a mean postoperative follow-up 4.6+/-1.9 years. The group of nine patients undergoing late correction had a mean age of 11.3+/-4.9 years (range 6.7-17.2 years), with a later surgical repair (mean age 4.7+/-2.7 years) for a moderate-sized ventricular septal defect (mean pulmonary/systemic output ratio 2.1/1; range 2.3-1.7) and a mean postoperative follow-up of 7+/-4.2 years. Each group of surgically repaired patients was compared with a control group matched for age, body surface area and gender. No significant differences were found between the normal controls and those undergoing early correction for any assessed functional index regarding left ventricular geometry (normalized volumes and mass for body surface area, mass/volume and thickness/radius ratios), shape (long axis-short axis ratio), diastolic (mitral and pulmonary venous flow patterns) and systolic (fractional shortening and rate-corrected mean velocity of circumferential fibre shortening) function. In addition, the data points for each patient for the rate-corrected mean velocity of circumferential fibre shortening to end-systolic stress relationship were within the 95% confidence limits of normal, suggesting normal left ventricular contractility. On the other hand, the patients undergoing surgery at a later age showed a persistent increase of the normalized left ventricular end-diastolic volume and mass, with an higher mass/volume ratio and reduced end-systolic stress compared with normal controls. Furthermore, left ventricular shape (long axis-short axis ratio) was abnormal at end-diastole but with its normal values at end-systole. Our data suggest that, in the presence of a large ventricular septal defect, early successful surgical repair <2 years of age results in complete recovery of left ventricular mechanics in the postoperative follow-up. In contrast, surgical closure at > 2 years of age, even for a moderately sized ventricular septal defect, deleteriously affects postoperative left ventricular geometry and shape. Since prolonged volume overload may be detrimental to myocardial function, earlier surgical repair should be recommended.

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.

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.

[Left ventricular systolic function in patients surgically treated for atrioventricular septal defect: echocardiographic assessment]. / Funzione sistolica ventricolare sinistra nei cav completi operati: valutazione ecocardiografica.

In order to assess the left ventricular systolic function we studied by echo 14 pts (8M, 6F) with complete atrioventricular septal defect (AVSD) who had undergone surgical repair. Mean age was 20.30 +/- 24.63 months, with a follow-up of 52.85 +/- 19.11%; in 6 pts Down syndrome was associated. Particularly, we tried to determine whether the following factors might affect the post-operative left ventricular systolic function: a) Down syndrome; b) residual mild mitral regurgitation; c) age of the surgical repair; d) length of the follow-up. As load-independent indexes of contractility, the left ventricular end systolic stress (LVESS)-circumferential fibre shortening velocity normalized for heart rate (VCFc) relationship and the LVESS/end systolic volume index (ESVI) ratio were chosen. All pts showed normal (mean +/- 2 standard deviations) or slightly higher values of LVESS/VCFc relationship; significantly, the only two pts with lower values had later undergone surgical repair. LVESS/ESVI ratio confirmed an inverse relationship between systolic function and age of the surgical correction (r = -0.75); no other factors (Down syndrome, residual mild mitral regurgitation, length of the follow-up) showed a significant correlation with the post-operative left ventricular systolic function. In conclusion, in our limited population, the age of the surgical repair appears to be the main factor affecting the post surgical left ventricular systolic function in pts with complete AVSD.

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.

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.

Cardiac valve replacement during pregnancy. Report of two cases.

Two successful cases of mitral valve replacement with cardiopulmonary by-pass and myocardial protection are reported. Postoperative anticoagulation was obtained with oral anticoagulants. The patients delivered two healthy newborns. The report suggests that an open-heart operation can be performed in pregnant women with an acceptable risk for the mother and the fetus.