Right ventricular function in congenital heart defects assessed by regional wall motion.

OBJECTIVES: To develop a simple method to assess right ventricular function by angiography. BACKGROUND: Conventional methods of evaluating right ventricular function are inaccurate, cumbersome, and expensive. METHODS: We analyzed biplane right ventricular angiograms taken in the posterior-anterior and lateral projections using software to measure right ventricular volumes and regional wall motion in 78 patients with normal hearts (n = 29), atrial septal defects (ASD n = 13), pulmonary valve stenosis (PVS n = 21), and postoperative atrial switch patients (n = 15). We also measured the shortening fraction (SF) from the midtricuspid annulus to the septum and correlated various angiographic measurements with the right ventricular (RV) ejection fraction. RESULTS: The volume-overloaded patients (ASD) had larger end diastolic volumes and increased SF compared with normal patients, while the pressure-loaded patients (PVS) had normal volumes and SF. The postoperative atrial switch patients had decreased systolic function and increased end diastolic volume. The SF for all of the patients correlated with the ejection fraction (r= 0.785, P

Simultaneous stent implantation to treat bifurcation stenoses in the pulmonary arteries: Initial results and long-term follow up.

BACKGROUND: Balloon angioplasty of bifurcating pulmonary artery (PA) stenoses is often inadequate, and stent treatment often requires simultaneous implantation of two stents. This study evaluates initial results and long-term follow up of transcatheter stent placement in bifurcating PAs. METHODS: This is a retrospective review of patients (pts) who had bifurcating PA stents placed in main and lobar branches from 1993 to 2007. RESULTS: Forty-nine pts had bifurcating PA stents placed at a median age of 10.9 years (range 1-43 years). The mean minimum vessel diameter increased from 5.7 +/- 2.5 mm to 11.0 +/- 3.6 mm (P < 0.001), the mean gradient across the stenoses decreased from 37.0 +/- 26.9 to 9.2 +/- 13 mm Hg (P < 0.001), whereas the mean RV:FA ratio decreased from 0.76 +/- 0.29 to 0.53 +/- 0.24 (P < 0.001). There was one death due to severe pulmonary hemorrhage. F/U data were available in 38 pts (mean duration 6.3 +/- 4.1 years, range 1.2-13.1 years). Thirty pts underwent repeat catheterizations (mean 2.3 +/- 2.2 years poststent), with 26 requiring further interventions: Fifteen had balloon angioplasty alone and 11 had additional stents placed. There were no complications at f/u catheterization. Six pts underwent further palliative surgeries, although none for repair of branch PA stenoses. CONCLUSIONS: Simultaneous transcatheter placement of bifurcating PA stents provides immediate gradient relief of bifurcating stenoses in the proximal or lobar branch PAs and reduces RV systolic pressure. Further interventions can be safely performed in future procedures, and the presence of stents does not complicate future surgeries.

Isolated cleft of the mitral valve: clinical spectrum and course.

We have reviewed the clinical presentations, courses, and outcomes of 90 patients seen at Texas Children's Hospital from 1983 through 2008 who had an isolated cleft of the mitral valve without some form of endocardial cushion defect. Additional congenital cardiac defects were present in 61 of the 90 patients, 35 of whom had a congenital syndrome. Seven patients had isolated cleft of the mitral valve without other intracardiac defects, and in these 7 there was a progressive increase in the degree of mitral regurgitation during a median time of 26.5 months from diagnosis to surgery. The patients ranged from a gestational age of approximately 32 weeks to 21.9 years of age. No death was observed among the 39 surgical patients, including 32 who had additional cardiac defects. There was a significant reduction in the degree of mitral regurgitation in all patients who underwent surgery. Among the 51 patients who did not have surgery, the degree of regurgitation did not change significantly over the course of 1 to 27 years' observation. Isolated cleft of the mitral valve is an uncommon (but not rare) congenital malformation of the mitral valve that can cause all degrees of mitral regurgitation but can be managed medically or surgically.

Atrial septostomy improves survival in select patients with pulmonary hypertension.

BACKGROUND: Atrial septostomy is a palliative treatment for patients with pulmonary hypertension (PHTN) refractory to vasodilator therapy. Limited data exist in the pediatric population and in patients with repaired congenital heart defects. METHODS: We performed a retrospective analysis of hemodynamic and symptomatic changes in patients with PHTN who underwent an atrial septostomy at our institution. RESULTS: Forty-six atrial septostomies were performed on 43 patients. Patient ages ranged from 0.3 to 30 years (median 12.5). Of 43 patients, 29 had primary PHTN, 10 had PHTN associated with repaired congenital heart defects, and 4 had other secondary causes of PHTN. Mean baseline pulmonary vascular resistance was 35 +/- 17 Wood units, and mean pulmonary artery pressure was 74 +/- 19 mm Hg. Patients surviving > or = 30 days had immediate improvement in cardiac index (from 2.3 to 2.9 L x min(-1) x m(-2), P < .0001), right atrial pressure (RAp) (from 9.9 to 8.3 mm Hg, P < .05), and oxygen delivery (from 424 to 491 mL O2 per minute, P < .01), with a decrease in systemic oxygen saturation (from 93% to 86%, P < .001). Pulmonary artery pressure was unchanged (P = .3). New York Heart Association class and symptoms of syncope improved (P < .01). Event-free survival at 1, 2, and 3 years was 84%, 77%, and 69%, respectively. Using the National Institutes of Health Registry model, predicted survival probability significantly improved (P < .001). Ten patients (22%) died within 30 days of catheterization. Mortality was associated with preceding decompensations in the intensive care unit (6/10, P < .001) and a higher RAp (21.4 vs 9.8 mm Hg, P < .001). CONCLUSIONS: Atrial septostomy provides symptomatic and hemodynamic improvement in cardiac index and RAp. Risk of septostomy is increased in patients with preceding decompensation or a RAp > 18 mm Hg.

Clinical presentation of persistent 5th aortic arch: 3 new cases.

We report 3 cases of a persistent 5th aortic arch. This anomaly is usually associated with other intracardiac defects. Although all 3 patients were found to have similar vascular structures that were best explained by the persistence of the 5th aortic arch into postnatal life, the clinical presentations were quite different. One patient presented with coarctation of the aorta, the 2nd patient with cyanosis, and the 3rd patient with pulmonary overcirculation. The 2nd case is unique in that the 5th aortic arch was found to be sensitive to prostaglandin E1. Understanding the embryologic derivation and differing presentations of a persistent 5th aortic arch aids in diagnosis and management of these patients.

Complex pulmonary atresia in a neonate: successful surgical palliation for right pulmonary artery arising from the right main coronary artery.

We describe the case of a 3-week-old infant who was diagnosed with pulmonary atresia, a ventricular septal defect, and discontinuous pulmonary arteries. The pulmonary artery anatomy was delineated by angiography: the right pulmonary artery arose from the proximal right coronary artery, and the left pulmonary artery arose from a left ductus arteriosus. This case illustrates the importance of careful delineation of the anatomy in complex pulmonary atresia before surgical intervention.

Cardiac catheterisation in infants weighing less than 2500 grams.

OBJECTIVES: To investigate the indications for, and outcome of, cardiac catheterisation in infants weighing less than 2500 g at a single institution over an 8-year period. PATIENTS AND METHODS: We assessed all infants who were less than 2500 g at the time of cardiac catheterisation at Texas Children's Hospital from January 1993 to January 2001. Comparisons of morbidity and mortality were drawn with an equivalent number of infants of similar age weighing greater than 2500 g seen over the same period of time. RESULTS: We performed interventional procedures in 22, and diagnostic catheterisations in 12 infants weighing less than 2500 g. Interventions included pulmonary valvoplasty in six patients, balloon angioplasty of critical coarctation in one, aortic valvoplasty in two, septostomy in ten, and coil occlusion of an arteriovenous malformation, redirection of a subclavian venous line, and coil occlusion of a patent arterial duct in one patient each. The median age at catheterisation was 5 days for children less than 2500 g, and 10 days for those above 2500 g. The median weights were 2.3 kg and 3.3 kg, and the median gestational ages were 35 weeks and 38 weeks, for the two respective groups. Of those weighing less than 2500 g, two died (6%), with no deaths occurring in those weighing more than 2500 g. In 3 patients weighing less than 2500 g (9%), there was vascular compromise, one child with bilateral femoral venous obstruction requiring fasciotomy compared, to one in the group weighing greater than 2500 g (2%). CONCLUSION: There is a significantly increased risk of mortality and vascular compromise in infants weighing less than 2500 g. Interventional catheterisation in these infants may be lifesaving, but given the aforementioned risks, diagnostic catheterisation should be deferred if possible in favor of noninvasive modalities.

Initial experience with intratherapeutics Intrastent Doublestrut LD stents in patients with congenital heart defects.

Limitations of the currently available balloon-expandable stainless steel (BE-SS) stents for use in patients with congenital heart defects (CHD) include inflexibility, significant shortening with expansion, sharp ends, and limited sizes. These limitations increase risk and greatly effect success of stent placement in these patients. The recently approved IntraStent DoubleStrut LD (IS-LD) stents are BE-SS stents designed to address such limitations. We report our initial experience with IS-LD stents in patients with CHD. Using standard techniques, 36 stents were implanted in 22 patients whose median age was 11 years (range, 1.4-35 years) and weight was 33 kg (range, 9-96.8 kg). Lesions stented included aortic coarctation (4), branch pulmonary arteries (19), inferior or superior vena cava (11), Mustard baffle (1), and Fontan baffle (1). All attempts at stent placement were successful. Hemodynamic assessment and angiography was performed pre- and poststent placement in all patients. Intravascular ultrasound was performed in four patients. Stenosis diameter increased from 5.7 +/- 0.6 mm (mean +/- SD) to 11.6 +/- 0.5 mm (P < 0.001). Stents did not shorten significantly with dilation (median percent shortening, 3.8%; P > 0.1). Complications included stent recoil (2), stent migration (1), and stent distortion (1). IS-LD stents are a safe and effective treatment for the majority of vascular stenoses in patients with CHD. Stent recoil at large diameters may limit its usefulness in selected lesions. Follow-up studies are required to determine the long-term performance of IS-LD stents.

Refinements in the implantation of pulmonary arterial stents: impact on morbidity and mortality of the procedure over the last two decades.

INTRODUCTION: There is limited data on medium to long-term outcome, and the morbidity and mortality associated with the implantation, of pulmonary arterial stents. PURPOSE: To assess changes in morbidity and mortality over the last two decades. METHODS: Retrospective analysis of all patients stented between September, 1989 and July, 2001. RESULTS: We implanted 664 Palmaz stents in 338 patients. The overall number included 229 patients who had undergone repair of tetralogy of Fallot, in whom 468 stents were implanted, 61 patients with congenital stenosis of the branches of the pulmonary trunk, in whom we placed 115 stents, 16 patients after an arterial switch operation who had 38 stents, and 32 patients after the Fontan operation who had 43 stents implanted. The mean age was 12.2 years, and the mean weight was 38 kg. The mean systolic pressure gradient decreased from 41 to 8.7 mmHg, the mean diameter of the stented vessel increased from 5.4 to 11.2 mm, and the ratio of right ventricular to femoral arterial pressure decreased from 0.66 to 0.45, each of these being significant at the level of p being less than 0.01. At a mean follow-up of 5.6 years, the mean gradient was 20 mmHg, the mean ratio of pressure between right ventricle and femoral artery was 0.5, and mean luminal diameter was 9.3 mm. Complications included migration of the stent in 8 patients, and pulmonary edema, hemoptysis and death in 5 patients each. There has been no mortality or morbidity since July of 1997. Technical changes include conservative serial dilations in congenital pulmonary arterial stenosis, avoidance of over-dilation, and simultaneous implantation of stents in the right and left pulmonary arteries in those with systemic pulmonary arterial pressure. Technological advances included shorter stents, improved balloon profiles, and central inflation of the stents. CONCLUSIONS: Modification of stenting practices, and increased experience of the operators over the last two decades, has virtually abolished any morbidity or mortality associated with the implantation of stents for congenital or postoperative pulmonary arterial stenoses.