Left ventricular dysfunction after long-term right ventricular apical pacing in the young.

OBJECTIVES: The goal of this study was to assess long-term global left ventricular (LV) function in patients paced from the right ventricular (RV) apex at a young age. BACKGROUND: Ventricular contraction asynchrony with short-term RV apical pacing has been associated with reduced LV pump function and relaxation. The long-term effect of RV apical pacing on global LV function in the young remains unknown. METHODS: Twenty-four patients with normal segmental anatomy paced from the RV apex (follow-up 1 to 19 years) underwent noninvasive assessment of global LV function with automated border detection echocardiography-derived fractional area of change (FAC), coupled with the Doppler index of myocardial performance (MPI). Data were analyzed from 24 RV-paced patients (mean follow-up 9.5 years, age 19 years, body surface area [BSA] 1.6 m2, QRS duration 140 ms) and compared with 33 age- and BSA-matched control subjects (age 16.4 years, BSA 1.6 m2). Multiple linear regression analysis was performed to identify patient variables that can affect these indexes of LV function. RESULTS: Assessment of LV function (median follow-up 10 years) in 24 paced patients demonstrated impaired area- and Doppler flow-derived indexes of LV systolic and diastolic function, compared with those indexes of control subjects (FAC: 52% vs. 60%, p < 0.01; MPI: 0.46 vs. 0.34, p < 0.01). Paced QRS interval and age were found to significantly influence global LV contraction in these patients (R2 = 0.4, p < 0.05). CONCLUSIONS: In the presence of impaired LV function with long-term RV apical pacing, alternative sites of ventricular pacing that simulate normal biventricular electrical activation should be explored to preserve function in pediatric patients in need of long-term pacing.

Aortic root dilation after the Ross procedure.

This study evaluated changes in neoaortic root geometry in patients who underwent the Ross procedure. Serial postoperative echocardiographic measurements of the neoaortic root indexed to the square root of body surface area (centimeters divided by meters) were obtained from 30 patients (age range 3.1 to 31.4 years) and compared with paired preoperative and immediate postoperative values. Normal aortic root diameter Z scores were derived from root dimensions obtained from 217 healthy controls. Compared with preoperative values, an immediate stretch of the neoaortic versus pulmonary root (annulus and sinuses of valsalva) was observed at a mean follow-up period of 1 week. Additional aortic annular dilation from baseline prehospital discharge values was observed at 2 to 12 months (baseline vs follow-up annulus Z score: 1.4 vs 2.6, p <0.01, n = 16) and at 16 to 33 months follow-up (0.8 vs 2.0, p <0.05, n = 12). In a similar fashion, there was additional enlargement of the aortic sinus from its stretched state at hospital discharge at 2 to 12 months (baseline vs follow-up sinus Z score: 2.0 vs 3.3, p <0.01, n = 17) and at 16 to 33 months (1.7 vs 3.0, p <0.01, n = 13). There were no differences in root size between 2 to 12 and 16 to 33 months after surgery. There was a decrease in left ventricular size with no alteration in blood pressure or degree of aortic valve regurgitation. Thus, aortic root dilation occurs up to the first year after the Ross procedure but does not appear to progress beyond this time.

Echocardiographic abnormalities with acute cardiac allograft rejection in children: correlation with endomyocardial biopsy.

In patients who have undergone successful orthotopic heart transplantation, echocardiography has the potential to be a noninvasive method for rejection surveillance that would reduce the frequency and guide the timing of endomyocardial biopsies, as well as allow for more frequent monitoring, with less cost and risk to the patient. To determine the applicability of echocardiography to detect moderate to severe rejection in children, 26 two-dimensional-guided M-mode echocardiograms with Doppler/color flow mapping were performed within 24 hours of endomyocardial biopsy. M-mode echocardiograms of the left ventricle were digitized and analyzed with a computer-assisted measurement format for left ventricular size, mass, and wall motion in systole and diastole. These echocardiographic parameters were clustered and analyzed by a unique echocardiographic scoring algorithm blinded to the biopsy interpretation. In eight cases in which findings of biopsies were consistent with moderate to severe rejection, left ventricular mass was increased and indexes of systolic and diastolic function were depressed compared with the remainder of the cases (n = 18), in which findings of biopsies included either no evidence or mild evidence of rejection. The echocardiographic score of the group with moderate to severe rejection was significantly greater than the score of the group that was normal or had mild rejection (5.4 +/- 0.7 vs 2.2 +/- 0.3; p < 0.001). With rejection prospectively defined as an echocardiographic score of greater than or equal to score 4, echocardiography achieved 88% sensitivity and 83% specificity in detecting moderate to severe rejection.(ABSTRACT TRUNCATED AT 250 WORDS)

Bidirectional cavopulmonary anastomosis with intracardiac repair.

BACKGROUND: Patients with hypoplasia of the right ventricle and tricuspid valve have historically undergone a total cavopulmonary connection or a two-ventricle repair with atrial fenestration. METHODS: We reviewed our experience with 9 patients with hypoplasia of the right ventricle and tricuspid valve who underwent a bidirectional cavopulmonary anastomosis with intracardiac repair. Patient diagnoses included pulmonary atresia with intact ventricular septum (n = 3); hypoplastic right ventricle and tricuspid valve with atrial septal defect, ventricular septal defect, and right ventricular outflow tract obstruction (n = 3); unbalanced atrioventricular canal defect (n = 1); inlet ventricular septal defect with coarctation (n = 1); and tricuspid stenosis with atrial septal defect (n = 1). RESULTS: The median age at operation was 36 months. There was hypoplasia of the right ventricle and tricuspid valve in all patients. The tricuspid valve measured 56.5% of normal (range, 43.6% to 70.4%) by echocardiography, and the median ratio of the tricuspid valve to the mitral valve was 0.67 (range, 0.54 to 0.82). At operation, the median tricuspid valve annulus diameter was 65.6% of published autopsy normals (range, 57.8% to 78.5%) with a median Z value of -3.8 (range, -6.6 to -2.1). All patients survived operation. At a median follow-up of 16 months, 5 patients are asymptomatic, and 2 have occasional early-morning periorbital edema. Two patients are on a regimen of diuretics, 1 of whom is also taking an unloading agent. The patient with unbalanced atrioventricular canal died suddenly at home 6 months postoperatively. CONCLUSIONS: Bidirectional cavopulmonary anastomosis with intracardiac repair may avoid the long-term complications associated with the Fontan modifications and eliminates the need of atrial fenestration in most instances. This operation should be considered for select patients with hypoplasia of the right ventricle and tricuspid valve.

A systematic approach to echocardiographic image acquisition and three-dimensional reconstruction with a subxiphoid rotational scan.

Rotational scanning from the subxiphoid position is an image acquisition technique used for reconstruction of dynamic three-dimensional echocardiographic images in infants and small children. The orientation of the heart within the three-dimensional data set is variable and dependent on the image plane at which rotational scanning was initiated. We describe an image acquisition technique that standardizes the orientation of the heart within the three-dimensional data set, thereby permitting a systematic approach to the reconstruction of three-dimensional renderings. Thirteen infants and small children with congenital heart disease were studied by this approach. Illustrative examples are provided. The average time required to derive a three-dimensional rendering was 37 +/- 9 minutes. We conclude that subxiphoid rotational scanning by a systematic approach to image acquisition and reconstruction can be applied successfully to the derivation of three-dimensional renderings of congenital cardiac defects.

Biventricular pacing as alternative therapy for dilated cardiomyopathy associated with congenital heart disease.

Biventricular, alternative, and multisite pacing are currently being explored to improve cardiac function among patients with medically refractory, end-stage dilated cardiomyopathies. Although, due to inherent myocardial abnormalities, patients with repaired congenital heart defects may be at a greater risk than others to develop heart failure, often requiring cardiac transplantation. The efficacy of biventricular pacing among these patients is unknown. This report presents a patient with successfully repaired congenital heart disease in infancy who developed a symptomatic dilated cardiomyopathy at 22 years of age. Following biventricular pacing, systemic ventricular function showed a 14% improvement in ventricular dP/dt. One month later, subjective symptoms improved and cardiac ultrasound illustrated a 125% increase in fractional area of change. Exercise stress testing showed a 17% improvement in aerobic work capacity.

Acute rheumatic fever in Wisconsin.

Medical records of patients discharged from Children's Hospital of Wisconsin from January 1980 to May 1988 who fulfilled the revised Jones criteria for acute rheumatic fever were reviewed. A total of 13 patients were hospitalized with a first attack of acute rheumatic fever during this period. Migratory polyarthritis was present in 92% of the patients and carditis in 62%. Mitral regurgitation was present in all cases of carditis. Only six patients (46%) had a history of a preceding streptococcal infection, half of whom were treated with antibiotics. While there is no evidence to support a new outbreak of acute rheumatic fever in Wisconsin, our data demonstrate that the disease is still prevalent within the eastern region of the state. Patients with Group A streptococcal pharyngitis must be identified and treated to prevent this disease from increasing in incidence.

Dynamic three-dimensional echocardiographic reconstruction of congenital cardiac septation defects.

Dynamic three-dimensional echocardiographic reconstructions of 27 cardiac septation defects were performed in 19 sedated infants and children. Using a subxiphoid rotational scanning approach, complete visualization of the entire shape and breadth of the defect was attained in 11 of 16 ventricular septal defects and 9 of 11 atrial septal defects. This technique enabled the operator to cut slices from the three-dimensional block of echocardiographic data in order to present septation defects in a region- or lesion-oriented fashion. Poor baseline two-dimensional image quality and certain small septation defects that were readily obscured by nearby structures led to inadequate three-dimensional reconstructions. The application of dynamic three-dimensional echocardiography for assessment of cardiac septation defects uniquely provided (1) the ability to present en face views of atrial and ventricular septal defects; and (2) a means by which intracardiac anatomy can be displayed in a region- and lesion-oriented fashion for interventional and surgical planning purposes.

Enhanced resting left ventricular filling in patients with successful coarctation repair and exercise-induced hypertension.

M-mode and Doppler echocardiographic analyses of left ventricular (LV) shortening and filling were performed in 50 patients who underwent coarctectomy (median follow-up 9.5 years) and in 16 athletes in a control group before an exercise stress test with upright bicycle ergometry was performed. Thirty-two of 50 patients and 18 of 50 patients had a normotensive and hypertensive response to exercise, respectively. Preexercise echocardiographic data were compared among the control, normotensive, and hypertensive patient groups. LV peak filling rates (dD/dt, diastole) were increased in the hypertensive group (18.3 +/- 3.5) compared with those in the normotensive group (14.4 +/- 3.2; p < 0.001) and the control group (13.6 +/- 2.8; p < 0.001). LV shortening was enhanced in the coarctectomy group compared with that in the control group. A higher aortic isthmus Doppler gradient at peak exercise was not found in the hypertensive group compared with that in the normotensive group. Therefore patients with successful coarctectomy in childhood have enhanced LV shortening and relaxation at rest. Demonstration of enhanced LV peak filling rates may help identify patients at risk for exercise-induced hypertension.

Preservation of endothelial cell structure and function by intracoronary perfluorochemical in a canine preparation of reperfusion.

To determine the effect of intracoronary perfluorochemical on endothelial cell structure and function, 16 dogs were randomized to receive either low-dose (15 ml/kg) intracoronary perfluorochemical (Fluosol-DA) or saline after 90 min of proximal occlusion of the left anterior descending coronary artery (LAD). The animals underwent reperfusion for 60 min with the introduction of perfluorochemical or saline 5 to 10 min after the onset of reperfusion. Endothelium-dependent coronary vasodilatory reserve was determined in vivo both at baseline and 1 hr after reperfusion by infusion of acetylcholine and then serotonin into the distal LAD bed in 12 animals (six in each group). Both agonists significantly increased regional flow measured by 133Xe washout in the two groups before occlusion, but at 1 hr after reperfusion only animals given perfluorochemical demonstrated a significant increase in flow. Vasodilatory reserve was assessed in vitro with cumulative dose-response curves to acetylcholine on LAD rings proximal and distal to the snare in all animals. These studies demonstrated a significant reduction in endothelial cell-mediated relaxation of epicardial arterial segments in the ischemic segment of control but not treated animals. Light microscopy revealed the presence of neutrophils within vessels in the ischemic zones in control animals only. Electron microscopy showed capillary obstruction by endothelial cell protrusions and neutrophil and red cell plugging in control animals in the ischemic region but an intact endothelium and predominantly unobstructed capillaries in treated animals. These findings suggest that the structural and functional endothelial damage after reperfusion may be prevented by the administration of intracoronary perfluorochemical after the onset of reperfusion.