Left ventricular diastolic filling in children with hypertrophic cardiomyopathy: assessment with pulsed Doppler echocardiography.

Altered left ventricular filling patterns in hypertrophic cardiomyopathy have been demonstrated by M-mode echocardiographic and radionuclide techniques. Because pulsed Doppler ultrasound provides the capability to directly measure blood flow velocity across the mitral valve, it was hypothesized that this technique would be useful for demonstrating left ventricular filling abnormalities. Simultaneous Doppler ultrasound examination of the left ventricular inflow, M-mode echocardiograms and phonocardiograms were performed in 17 children and young adults: 10 with hypertrophic cardiomyopathy (aged 6 to 20 years) and 7 with a normal heart (aged 10 to 18 years). From the Doppler studies, measurements of various diastolic time intervals, peak flow velocity during rapid filling (E velocity) and peak flow velocity during atrial contraction (A velocity) were made. Several areas within the Doppler flow envelope were calculated: first 33% of diastole (0.33 area), first 50% of diastole, triangle under the E velocity (E area) and triangle under the A velocity (A area). These were expressed as a percent of area under the total flow envelope. From the M-mode studies, left ventricular endocardial echoes were digitized and peak rates of increase in left ventricular dimension were determined and normalized for end-diastolic dimension. Diastolic time intervals, including isovolumic relaxation time, were calculated using the phonocardiogram to determine end-systole. The E velocity was lower (0.71 +/- 0.23 versus 0.91 +/- 0.11 m/s, p less than 0.05), 0.33 area/total area was less (0.46 +/- 0.11 versus 0.58 +/- 0.08, p less than 0.05) and the isovolumic relaxation time was prolonged (56 +/- 2 versus 31 +/- 1 ms, p less than 0.05) in patients with hypertrophic cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Mucocutaneous lymph node syndrome (Kawasaki disease): delayed aortic and mitral insufficiency secondary to active valvulitis.

A 2 8/12 year old girl with acute mucocutaneous lymph node syndrome (Kawasaki disease), presented 17 months later with a new onset of mitral and aortic insufficiency. Congestive heart failure rapidly developed and double valve replacement was performed. Pathologic study of the excised valve tissue disclosed active valvulitis. This is the first report of late onset aortic and mitral valvulitis associated with Kawasaki disease.

Cardiac function in smokers and nonsmokers: the CARDIA study. The Coronary Artery Risk Development in Young Adults Study.

OBJECTIVES: This study assessed clinical and echocardiographic measures of cardiac function at rest in smokers and nonsmokers to determine the associations of cigarette smoking with various measures of left and right ventricular performance. BACKGROUND: Whereas the immediate cardiovascular effects of cigarette smoking have been well described, the long-term effects in an otherwise healthy cohort have not. Of particular interest were associations with heart rate, left ventricular end-systolic stress and left ventricular mass because higher levels of these measures would suggest increased myocardial oxygen consumption. METHODS: In year 5 of the Coronary Artery Risk Development in Young Adults (CARDIA) study, 3,366 smokers and nonsmokers (ex-smokers were excluded) underwent echocardiography as well as assessment of heart rate, anthropometric measurements and blood pressure. Participants ranged in age from 23 to 35 years and were equally distributed by race and gender. Echocardiographic measures included pulsed Doppler pulmonary artery acceleration time (a decrease suggests increased pulmonary artery pressure), left ventricular mass, left ventricular end-systolic stress and left ventricular fractional shortening. RESULTS: All comparisons were between smokers and nonsmokers. Heart rate at rest was significantly higher in smokers by 1.5 to 5 beats/min in all race/gender groups except black men. In men who smoked, pulmonary artery acceleration time was significantly lower by 4 to 8 ms. Except for black male smokers, there was a trend toward increased left ventricular mass (3 to 8 g) in all race/gender groups, significant in black women. Left ventricular end-systolic stress was significantly higher in women who smoked (4 to 6 dynes/cm2). There were no differences for systolic blood pressure or left ventricular fractional shortening. CONCLUSION: In an assessment of cardiovascular function at rest in young adults, quantifiable differences between smokers and nonsmokers that predict increased rest myocardial oxygen consumption in smokers were found. Some of these differences were gender specific.

Fetal tachycardia: mechanisms and predictors of hydrops fetalis.

OBJECTIVES: This study had three objectives: 1) to determine the electrophysiologic mechanisms of fetal supraventricular tachycardia at presentation and postnatally; 2) to identify the clinical and electrophysiologic predictors of hydrops fetalis; and 3) to describe the medium-term follow-up (1 to 7 years) of patients with fetal supraventricular tachycardia. BACKGROUND: Fetal supraventricular tachycardia causes significant fetal and neonatal morbidity and mortality. Prenatal analysis and postnatal confirmation of fetal supraventricular tachycardia mechanisms have been limited. METHODS: Supraventricular tachycardia mechanisms were evaluated by prenatal Doppler/M-mode echocardiography, immediate neonatal surface electrocardiography and postnatal transesophageal electrophysiologic procedures in 30 consecutive patients presenting with fetal supraventricular tachycardia (17 managed prenatally, 13 first managed postnatally). RESULTS: The fetal supraventricular tachycardia mechanism was 1:1 atrioventricular conduction in 22 patients and supraventricular tachycardia with atrioventricular block (atrial flutter) in 8. At the postnatal transesophageal electrophysiologic procedure, tachycardia was induced in 27 of 30 patients; atrioventricular reentrant tachycardia in 25 (93%) of 27 and intraatrial reentrant tachycardia in only 2 (7%) of 27. Hydrops was present in 12 of 30 fetuses. Sustained supraventricular tachycardia (> 12 h) and lower gestation at presentation correlated with hydrops (p < 0.02, p < 0.05), but mechanism of tachycardia and heart rate did not. Gestational age at delivery was significantly greater in those who received intrauterine management (39 +/- 1.3 vs. 37 +/- 2.9 weeks, p = 0.04) despite earlier presentation (32.6 vs. 37.1 weeks). Cesarean section deliveries were reduced in the same group (3 of 17 vs. 11 of 13, p = 0.0006). CONCLUSIONS: Atrioventricular reentrant tachycardia was the predominant mechanism of supraventricular tachycardia in the fetus. There was a high association of supraventricular tachycardia with atrioventricular block in utero and accessory atrioventricular connections. Outcome at 1 to 7 years was excellent regardless of severity of illness at clinical presentation.

Day-to-day variability of serum cholesterol, triglyceride, and high-density lipoprotein cholesterol levels. Impact on the assessment of risk according to the National Cholesterol Education Program guidelines.

The National Cholesterol Education Program has recently published guidelines for the assessment of cardiovascular risk and goals for laboratory accuracy. To test the impact of biologic and analytic variability on the ability of a single lipid measurement to assess risk accurately, lipids were measured on three occasions in 51 volunteers. Notable day-to-day variability of total cholesterol (5%), triglyceride (20%), high-density lipoprotein cholesterol (10%), and calculated low-density lipoprotein cholesterol (8%) levels was found. Analytic variability contributed significantly to total variability of high-density lipoprotein cholesterol levels and calculated low-density lipoprotein cholesterol levels. Confidence intervals constructed around National Cholesterol Education Program cutoff points suggested that classification was reliable from a single measurement if total cholesterol value was below 4.78 (less than 185 mg/dL), between 5.56 and 5.81 (215 and 225 mg/dL), or above 6.59 mmol/L (greater than 225 mg/dL). Low-density lipoprotein cholesterol value classification from a single measurement was only accurate at below 3.00 (greater than 116 mg/dL) or above 4.50 mmol/L (greater than 174 mg/dL). This study documents significant day-to-day variability of serum lipids and suggests that patients near the National Cholesterol Education Program cutoff points may require repeated measurements to assign risk accurately.

Echocardiographic assessment of cardiac function during respiratory syncytial virus infection.

Respiratory syncytial virus infection has been associated with increased morbidity and mortality in infants with underlying cardiac and pulmonary disease. To understand better the cardiopulmonary interaction in patients with acute respiratory syncytial virus bronchiolitis, we performed M-mode echocardiograms and pulsed Doppler assessment of pulmonary arterial flow in 19 patients with structurally normal hearts during acute illness. Studies were repeated in 11 of these patients following complete recovery. Based on severity of respiratory compromise, patients were grouped into those with severe illness (ten patients) or mild illness (nine patients). Left ventricular dimensions and shortening fraction were used to assess left ventricular function. Right ventricular systolic time intervals and specific Doppler flow velocity measurements were used to assess right ventricular function and elevation of pulmonary artery pressure. Comparisons were made between patients with severe and mild illness and between acute and follow-up studies. No statistically significant differences in left ventricular function, right ventricular systolic time intervals, or Doppler flow measurements were observed. We conclude that in patients with structurally normal hearts, respiratory syncytial virus bronchiolitis is not associated with significant depression of cardiac performance or elevation in pulmonary resistance.

Effect of iron deficiency on tissue oxygen delivery in cyanotic congenital heart disease.

To test the hypothesis that tissue oxygen delivery would be affected by diminished oxygen stores in cyanotic congenital heart disease, serum ferritin, transferrin saturation, hemoglobin, red cell mean corpuscular volume (MCV), red cell 2,3-diphosphoglycerate (DPG), P50, blood gases, oxygen saturations and systemic oxygen transport were measured in 29 hypoxemic infants and children. For the group, aortic saturation was 81 +/- 9%, PaO2 was 50 +/- 12 mm Hg, hemoglobin 16.2 +/- 2.1 gm/dl and systemic oxygen transport 620 +/- 145 ml/min/m2. P50 was increased above normal values (28.8 +/- 2.3 vs 26.6 +/- 1.1 mm Hg, p less than 0.01), and DPG was 2.35 +/- 0.54 mumol/ml, at the upper limits of normal for this assay. Iron deficiency was present in 8. When patients with P50 greater than or equal to 30 mm Hg and P50 less than 30 mm Hg were compared, iron stores were diminished in the high P50 group: [serum ferritin (19 +/- 8 vs 53 +/- 48 ng/ml, p = 0.0006), transferrin saturation (11 +/- 6 vs 23 +/- 11%, p = 0.003) and MCV (79 +/- 8 vs 86 +/- 4 fl, p = 0.05)]. Hemoglobin, aortic oxygen saturation, PaO2 and systemic oxygen transport were similar in both groups. In children with iron sufficiency, 15 of 21 had MCV greater than 90th percentile for age and sex (p less than 0.001 versus expected distribution). Also, MCV greater than 90th percentile for age and sex had a positive predictive value of 0.88 for iron sufficiency. This study demonstrates that diminished iron stores in cyanotic congenital heart disease are associated with a more right-shifted oxyhemoglobin dissociation curve (increased P50).(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormal systemic venous Doppler flow patterns in atrial tachycardia in infants.

Congestive heart failure due to atrial tachycardia in the neonate may be caused in part by altered preload and changes in diastolic flow characteristics. During atrial tachycardia, venous flow reversal is present. Onset of flow reversal is associated with a ventriculoatrial interval. Decreased tricuspid valve inflow mean velocity and time velocity integral, decreased diastolic duration, and delayed diastolic onset were present during tachycardia when compared with sinus rhythm.

Response to the Valsalva maneuver after the Fontan procedure for tricuspid valve atresia, single ventricle or pulmonic valve atresia.

The Valsalva maneuver was performed during cardiac catheterization in 9 patients who had undergone a Fontan operation (Fontan group) and in 10 control subjects. The Fontan group had higher right atrial (RA) pressure (16 +/- 1 vs 4 +/- 0.5 mm Hg, p less than 0.001), lower cardiac index (2.5 +/- 0.1 vs 3.7 +/- 0.2 liters/min/m2, p less than 0.05), lower stroke index (32 +/- 2 vs 44 +/- 2 ml/beat/m2, p less than 0.05), and higher systemic vascular resistance (31 +/- 1 vs 24 +/- 1 units, p less than 0.05) than the control group. Four patients in the Fontan group had a normal 4-phase Valsalva response, and 5 did not (p less than 0.05). RA pressure was similar in those who responded normally and those who did not (16 +/- 0.5 vs 17 +/- 1 mm Hg), whereas in those who responded normally cardiac index was higher (2.9 +/- 0.2 vs 2.2 +/- 0.1 liters/min/m2, p less than 0.05), stroke index was higher (36 +/- 1 vs 28 +/- 1 ml/beat/m2, p less than 0.05), and systemic vascular resistance was lower (28 +/- 1 vs 31 +/- 1, p less than 0.05). It is concluded that cardiac output is a better predictor of a normal response to the Valsalva maneuver than RA pressure in patients after the Fontan procedure. A normal response to the supine Valsalva maneuver suggests a cardiac index greater than 2.4 liters/min/m2 and stroke index greater than 31 ml/beat/m2.

Usefulness of echocardiographic evidence of pericardial effusion and mitral regurgitation during the acute stage in predicting development of coronary arterial aneurysms in the late stage of Kawasaki disease.

In 28 patients with Kawasaki disease, the relation of specific echocardiographic findings identified during the acute study of the illness, including valvular regurgitation, to development of coronary aneurysms was evaluated. Initial studies were performed at the time of clinical presentation, 5 to 10 days after the onset of fever, and follow-up studies were performed 1 to 2 months later. Patients in whom coronary aneurysms developed were more likely to have pericardial effusion (p = 0.0006) or mitral regurgitation (MR) (p = 0.014) at initial echocardiographic study than those without aneurysms. Presence of either mitral regurgitation (MR) or pericardial effusion had a positive predictive value of 0.84 for aneurysm development. Twenty-three percent of patients had MR, and it was associated with mild LV dilatation (35 +/- 3 vs 32 +/- 5 mm, p less than 0.05). Insufficiency of other valves was rare. Thus, MR and pericardial effusion on acute phase echocardiographic examination may predict development of coronary aneurysms in Kawasaki disease. Mild MR occurs frequently in acute Kawasaki disease and is associated with mild LV dilation.

Usefulness of parental serum total cholesterol levels in identifying children with hypercholesterolemia.

It was hypothesized that healthy children with high cholesterol levels may have parents who exceed acceptable cholesterol levels established by the National Cholesterol Education Program. One hundred sixty families (320 parents, 263 children aged 3 to 10 years) were evaluated for total cholesterol and other risk factors. Before the study, almost half of the parents had not had serum total cholesterol measured. The odds ratio for a child having a total cholesterol greater than or equal to 5.17 mmol/liter (200 mg/dl) was 13.6:1 (confidence interval 5.7 to 32.5) for a child with at least 1 parent having cholesterol greater than or equal to 6.20 mmol/liter (240 mg/dl) versus a child whose parents had low total cholesterol. Testing only children who had at least 1 parent with a total cholesterol greater than or equal to 5.17 mmol/liter (200 mg/dl) had a sensitivity of 98% for detecting children's total cholesterol greater than or equal to 5.17 mmol/liter. It is concluded that parental total cholesterol is useful in identifying children with high total cholesterol levels. Pediatricians may identify a large number of parents with hypercholesterolemia not previously recognized.

Reversal of severe late left ventricular failure after pediatric heart transplantation and possible role of plasmapheresis.

Late acute cardiac graft failure carries a high mortality in adults. Vascular mediators and factors other than classic T-cell-mediated rejection may play a role in this process, and aggressive multimodality therapy may improve survival. We report experience with plasmapheresis in treating late severe acute left ventricular dysfunction in a group of pediatric heart transplant recipients. We retrospectively reviewed clinical records, echocardiograms, hemodynamics, coronary angiograms, biopsy specimens, and treatment regimens for 5 patients with 7 episodes of late-onset severe graft failure who recovered. Plasmapheresis was applied in all cases, in addition to methylprednisolone, cyclophosphamide, lympholytic agents, and aggressive supportive care including mechanical ventilation and hemofiltration. All patients presented with acute severe left ventricular dysfunction 1.4 to 7.9 years (mean 3.6) after orthotopic heart transplantation. Mean shortening fraction at presentation was 13 to 23% (mean 16), initial endomyocardial biopsy specimens were grade 0 to 3B, and immunofluorescence studies were negative. Treatment included plasmapheresis, cyclophosphamide, mechanical ventilation, hemofiltration, and inotropes. Clinical recovery was slow, with 4 to 8 weeks until left ventricular function normalized, and 2.2 to 9.4 (mean 4.6) weeks to hospital discharge. At follow-up (50 to 38 months, mean 24), all are alive. Two patients are well, whereas coronary vasculopathy developed in 3. Thus, survival may improve in patients with late graft failure with low biopsy score and plasmapheresis combined with multimodality therapy.

Doppler evaluation of left ventricular diastolic filling in children with systemic hypertension.

To assess left ventricular (LV) diastolic function in children with systemic hypertension, 11 patients with hypertension (mean blood pressure 99 mm Hg) and 7 normal patients (mean blood pressure 78 mm Hg) underwent M-mode echocardiography and pulsed Doppler examination of the LV inflow. From a digitized trace of the LV endocardium and a simultaneous phonocardiogram, echocardiographic diastolic time intervals, peak rate of increase in LV dimension (dD/dt), and dD/dt normalized for LV end-diastolic dimension (dD/dt/D) were measured. Doppler diastolic time intervals, peak velocities at rapid filling (E velocity) and atrial contraction (A velocity), and the ratio of E and A velocities were measured. The following areas under the Doppler curve and their percent of the total area were determined: first 33% of diastole (0.33 area), first 50% of diastole, triangle under the A velocity (A area), and the triangle under the E velocity (E area). The A velocity (patients with hypertension = 0.68 +/- 0.11 m/s, normal subjects = 0.49 +/- 0.08 m/s), the 0.33 area/total area (patients with hypertension = 0.49 +/- 0.09, normal subjects = 0.58 +/- 0.08), the A area (patients with hypertension = 0.17 +/- 0.05, normal subjects = 0.12 +/- 0.03), and the A area/total area (patients with hypertension = 0.30 +/- 0.11, normal subjects = 0.20 +/- 0.07) were significantly different between groups (p less than 0.05). M-mode and Doppler time intervals, (dD/dt)/D, E velocity, and the remaining Doppler areas were not significantly different between groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Prevalence and identification of abnormal lipoprotein levels in a biracial population aged 23 to 35 years (the CARDIA Study). The Coronary Artery Risk Development in Young Adults Study.

This study examines the prevalence of abnormal low-density lipoprotein (LDL) and high-density lipoprotein (HDL) cholesterol levels in young adults to determine the ability of National Cholesterol Education Program Adult Treatment Panel (ATP) guidelines to identify persons with elevated LDL cholesterol, to compare other algorithms with those of the ATP, and to determine the contributions of race, gender, and other coronary artery disease risk factors to identifying patients with elevated LDL and low HDL cholesterol. The cohort was population-based, aged 23 to 35 years, and included relatively equal numbers of blacks and whites, and men and women. The prevalence of LDL cholesterol > or = 160 mg/dl (> 4.1 mmol/L) was 5% in black women, 4% in white women, 10% in black men, and 9% in white men. ATP identified most participants with elevated LDL cholesterol (range: 58.8% of white men to 70.7% of black women). Lipoprotein panels would have been required in 6% to 7% of women and to 15% to 18% of men. Algorithms that used nonlipid risk factors required more lipoprotein panels and identified fewer additional participants at risk. The prevalence of HDL cholesterol < 35 mg/dl (0.9 mmol/L) was 3% in women, 7% in black men, and 13% in white men. Algorithms that used nonlipid risk factors before measuring HDL cholesterol would require HDL cholesterol measurements in 35% of whites and 56% of blacks, but reduced sensitivity for identifying low HDL cholesterol (range: 58% in white men to 93% in black women). In young adults, algorithms based on nonlipid risk factors and family history have lower sensitivity, and increase rather than decrease the number of fasting lipoprotein panels required when compared with ATP levels.

Differences in cardiovascular disease risk factors in black and white young adults: comparisons among five communities of the CARDIA and the Bogalusa heart studies. Coronary Artery Risk Development In Young Adults.

PURPOSE: To examine community differences in cardiovascular disease (CVD) risk factors among black and white young adults by combining data from two large epidemiologic studies. METHODS: Data are from participants aged 20-31 years in the Coronary Artery Risk Development In Young Adults (CARDIA) study (1987-1988; N = 4129) and the Bogalusa Heart study (1988-1991; N = 1884), adjusting for data collection differences prior to analysis. CARDIA includes four urban sites: Birmingham, Alabama; Chicago, Illinois; Minneapolis, Minnesota; and Oakland, California. Bogalusa is a semi-rural town in Southeastern Louisiana. CVD risk factors examined were smoking status, body habitus, and blood pressure. RESULTS: In Birmingham and Bogalusa, more white than black women were current smokers; no ethnic differences were observed among men. In Chicago, Minneapolis, and Oakland, more blacks were current smokers than were whites. For all sites, educational level was strongly inversely related to current smoking status; ethnic differences were more apparent among those with up to a high school education. Among white men and women, prevalence of obesity (body mass index > 31.1 kg/m2 in men and 32.3 kg/m2 in women) was greater in Birmingham and Bogalusa than in Chicago. Minneapolis, and Oakland. Mean systolic blood pressures were highest in Bogalusa, and the proportion of black men with elevated blood pressure (> or = 130/85 mmHg) was higher in Bogalusa and Birmingham. CONCLUSIONS: Community and ethnic differences in CVD risk factors were observed among young adults in two large epidemiologic studies. Further studies may enhance our understanding of the relationship of geographic differences in CVD risk to subsequent disease.

Evolution of latent hypoparathyroidism in familial 22q11 deletion syndrome.

Latent hypoparathyroidism (LHP), the inability to increase midmolecular parathyroid hormone levels appropriately during a hypocalcemic challenge, was reported previously in an asymptomatic woman with tetralogy of Fallot. This women's fourth child died with DiGeorge anomaly. Seven years later, we restudied the index patient with LHP and evaluated three generations of her family for parathyroid dysfunction, cardiac abnormalities, and del 22(q11). Deletions were found in six relatives, three with conotruncal cardiac defects and three with a structurally normal heart. We found significant transgenerational noncardiac phenotypic variability, including learning difficulties, dysmorphic facial appearance, and psychiatric illness. A spectrum of parathyroid gland dysfunction associated with the del 22(q11) was seen, ranging from hypocalcemic hypoparathyroidism to normocalcemia with abnormally low basal intact parathyroid hormone (iPTH) levels. In addition, LHP found in the index patient 7 years ago had evolved to frank hypocalcemic hypoparathyroidism. In this family, which is the largest family with 22q11 deletions studied to date, parathyroid gland dysfunction evolved over time. We suggest that the calcium parathyroid hormone axis of unrelated patients with del 22(q11) be followed closely for the development of hypocalcemic hypoparathyroidism.

Airway compression by a right aortic arch in the absence of a vascular ring.

This case describes the diagnosis and successful management of an unusual form of respiratory distress in an infant with tetralogy of Fallot. Severe compression of the airway resulted from a large right aortic arch in the absence of a vascular ring.

Preparation of the left ventricle for anatomical correction in patients with simple transposition of the great arteries. Surgical guidelines.

Pulmonary artery banding in combination with an aortopulmonary shunt was performed on 16 patients with simple transposition of the great arteries to prepare the left ventricle for anatomical correction. Three groups were identified after operation: Group I (four patients) had increased pulmonary blood flow and tight pulmonary artery banding; Group II (four patients) had increased pulmonary blood flow and moderate pulmonary artery banding; Group III (eight patients) had normal pulmonary blood flow and moderate pulmonary artery banding. Postoperative low cardiac output was present in all patients in Group I, whereas mild heart failure was present in two patients in Group II and in two in Group III. There was one hospital death (6%). The follow-up period was 125 patient-months. Left ventricular systolic pressure rose from 63 +/- 11 torr before the operation to 101 +/- 35 torr after the procedure in Group I (p less than 0.05), from 59 +/- 10 to 93 +/- 33 torr in Group II (p less than 0.05), and from 55 +/- 10 to 84 +/- 16 torr in Group III (p less than 0.005). The increase in left ventricular muscle mass was from 44 +/- 2 gm/m2 preoperatively to 108 +/- 12 gm/m2 after operation in Group I (p less than 0.01), from 43 +/- 3 to 93 +/- 8 gm/m2 in Group II (p less than 0.02), and from 46 +/- 3 to 55 +/- 14 gm/m2 in Group III (p = no statistically significant difference). The postoperative change in left ventricular end-diastolic volume was from 100% +/- 17% to 133% +/- 23% of normal in Groups I and II (p less than 0.05) and from 123% +/- 29% to 107% +/- 36% of normal in Group III (p = no statistically significant difference). In preparing the left ventricle for anatomical correction, avoidance of severe pulmonary artery banding decreases the incidence of postoperative myocardial dysfunction, a moderate degree of volume overload and pulmonary artery banding provides the most effective stimulus for ventricular growth, and a small to moderate atrial septal defect is advantageous because it ensures the volume preload necessary for the development of the left ventricle.

Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. Surgical implications.

Postoperative cardiac catheterization data of 74 patients with pulmonary insufficiency after tetralogy repair were analyzed. Two groups were identified: Group A, 26 patients with normal right ventricular function (ejection fraction 95% +/- 5.5%, end-systolic volume 110% +/- 17% of predicted normal) and Group B, 48 patients with right ventricular dysfunction (ejection fraction 80% +/- 18% [p less than 0.001], and end-systolic volume 218% +/- 75% of predicted normal [p less than 0.001]). There was no significant difference between the two groups with respect to frequency of previous palliative procedures, age at operative repair, operative techniques, methods of myocardial protection, and follow-up period. Right ventricular dysfunction in Group B was associated with significant distal pulmonary stenosis (right ventricle-pulmonary artery pressure gradient 28 +/- 13 torr in Group A versus 55 +/- 20 torr in Group B, p less than 0.001), moderate pulmonary regurgitation (regurgitant fraction 18% +/- 11% in Group A versus 32% +/- 10% in Group B, p less than 0.001), and large transannular outflow patch (ratio of patch diameter to descending aorta diameter 1.31 +/- 0.16 in Group A versus 2.50 +/- 0.28 in Group B, p less than 0.001). Pulmonary valve insertion was performed in 42 patients in Group B. Eighteen had subsequent cardiac catheterization. Right ventricular function recovered completely (end-systolic volume 122% +/- 24%, and ejection fraction 92% +/- 7% of predicted) in five of six patients (83%) who had valve insertion within the first 2 years after tetralogy repair. In contrast, right ventricular function remained abnormal in all 12 patients who had valve insertion later than 2 years after tetralogy repair (p less than 0.05). Patients with residual pulmonary stenosis and/or a large transannular outflow patch are at risk for the development of right ventricular dysfunction from pulmonary insufficiency after tetralogy repair. Early correction of these residual lesions and control of pulmonary insufficiency may prevent long-term deterioration in right ventricular function.

Fontan type operation for complex lesions. Surgical considerations to improve survival.

Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used. Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previous pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 +/- 6.5 torr), and hospital stay longer (31 days). In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 +/- 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlusion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13%). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing well. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valve closure, avoidance of valved conduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.