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Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22-29 years).

BACKGROUND: Great concern exists about the ability of the anatomic right ventricle to sustain the systemic circulation in patients with transposition of the great arteries who have undergone a Mustard procedure. A prospective study was made to examine long-term survival, clinical outcome, and right ventricular function 25 years after surgery. METHODS: Ninety-one consecutive patients underwent the Mustard procedure between 1973 and 1980. After 14 years and again after 25 years (range 22-29 years), patients were studied with ECG, echocardiography, exercise testing, and Holter monitoring. RESULTS: The cumulative survival and event-free survival were 77% and 36%, respectively, after 25 years. Reoperation was necessary in 46%. No major loss of sinus rhythm was found. While all patients had good right ventricular function 14 years after repair, 61% of patients showed moderate-to-severe dysfunction after 25 years, when studied by echocardiography. Furthermore, the QRS complex widened and exercise capacity decreased. CONCLUSION: The anatomic right ventricle appears to be unable to sustain the systemic circulation at long-term follow-up and the clinical condition of patients late after Mustard repair is declining. We can expect more deaths or need for heart transplantation in the next decade.

Adult↗

Postsurgical course of patients with congenitally corrected transposition of the great arteries.

Because congenitally corrected transposition is a rare congenital anomaly, little is known about the prognosis of patients with this syndrome. The present study evaluated the functional status and clinical course of 73 patients (42 male and 31 female) aged 10 days to 73 years. Follow-up was 1 week to 37 years (mean 12.7 years). Particular attention was paid to the systemic right ventricle, considering the current controversies about long-term right ventricular function. Survival of patients with this condition in general was significantly below normal. The total mortality rate was 11% (8 patients) after a mean follow-up of 12.7 years (range 10 days to 37 years). Mean age at death was 18.5 years (range 6 days to 63 years). The mean age of the survivors at latest follow-up was 21.4 years (range 4 months to 73 years). In most patients, right ventricular function deteriorated and tricuspid valve regurgitation increased, which began at a very young age. Patients without associated lesions developed complications at a higher age compared with the total group. Right ventricular and tricuspid valvular function deteriorated more frequently in patients following intracardiac operation (28% and 52%, respectively) compared with patients undergoing palliative intervention (16%) or no surgery at all (28%). We conclude that survival of patients with this condition is substantially reduced compared with the natural history of an age- and gender-matched general population. This could be explained by the associated cardiac defects, but also by the anatomic condition itself.

Adolescent↗

Echocardiographic assessment of right and left ventricular function after single-lung transplantation.

To evaluate the impact of single-lung transplantation on right and left ventricular performance and to identify potential preoperative characteristics that could predict postoperative outcome, a large group of patients were retrospectively evaluated by means of serial Doppler echocardiography. Twenty-six of 57 consecutive single-lung transplant patients had satisfactory Doppler echocardiographic studies before and after surgery. A significant reduction in right ventricular diastolic and systolic areas and an increase in right ventricular fractional area were noted after transplantation. The left ventricular contractility remained unchanged. In addition, significant reduction in right atrial area, right ventricular free wall thickness, and tricuspid regurgitation were also seen. Paradoxical septal motion present in eight patients before the procedure resolved in all of them after single-lung transplantation. Transthoracic echocardiography is a useful technique to document improvement in right ventricular function in 63% of patients following single-lung transplantation. Preoperative paradoxical septal motion identifies single-lung transplant recipients who have the largest postoperative right ventricular area reduction and fractional area increase. Alternative imaging modalities should be sought for single-lung transplant candidates in whom conventional transthoracic echocardiography is suboptimal.

Adult↗

Cardiac autonomic impairment and early myocardial damage involving the right ventricle are independent phenomena in Chagas' disease.

Cardiac autonomic impairment and right side heart failure are prominent features in patients with Chagas' disease, but no causal relationship between these phenomena has been disclosed and the pathophysiology of such manifestations is unclear. Aim of study was to assess the cardiac autonomic control and biventricular function in chagasic patients in early stages of the disease, using radionuclide angiography, Valsalva manoeuvre, head-up tilt and baroreflex sensitivity evaluation. Thirty-one chagasic patients with no clinical signs of Chagas' heart disease-16 in the indeterminate phase and 15 with sole organic digestive involvement-were studied, and results compared with those obtained in 14 normal volunteers. No significant differences were observed among the three groups, in regard to any systolic or diastolic parameter of LV function, including ejection fraction, peak ejection and filling rates and correspondent times, time to end-systole, and the standard deviation of phase values. The indeterminate and digestive groups of chagasics had significantly lower right ventricular ejection fraction (45.7 +/- 6.3 and 46.2 +/- 10.1 respectively) and peak ejection rate (respectively 2.8 +/- 0.6 and 2.9 +/- 0.6) and higher right ventricular phase standard deviation (22.4 +/- 5.9 and 20.1 +/- 5.6 degrees, respectively), as compared with the control group (53.6 +/- 4.3, 3.5 +/- 0.5, and 15.8 +/- 3.8 respectively for right ventricular ejection fraction, peak ejection rate and phase standard deviation). No significant differences were found between the results of autonomic evaluation in the control and indeterminate groups of chagasic patients. The group of digestive disease patients showed abnormally lower Valsalva ratio (1.5 +/- 0.15), baroreflex sensitivity (8.85 +/- 2.05 ms/mmHg) and parasympathetically-dependent heart rate response to tilt (8.85 +/- 8.42 beats/mm) and higher Valsalva delay (15.67 +/- 1.35 s) values, compared with the control group (respectively 1.85 +/- 0.49, 20.23 +/- 12.66 ms/mmHg, 21.61 +/- 5.77 beats/mm and 10.1 +/- 2.5 s). Thus, cardiac autonomic impairment is a prominent feature in chagasic patients with the digestive but not the indeterminate form of Chagas' disease. It bears no causative relationship to the early myocardial damage that is apparent only regarding right ventricular function, in both groups of patients. Early right ventricular dysfunction is a likely mechanism for the marked predominance of systemic over pulmonary congestion when heart failure supervenes in patients with Chagas' disease.

Adult↗

Differentiation between primary dilated cardiomyopathy and ischemic cardiomyopathy based on right ventricular performance.

The differentiation of primary dilated cardiomyopathy from ischemic cardiomyopathy, though important, is difficult clinically and may require coronary angiography or metabolic imaging. Both patient groups have severe left ventricular dysfunction and severe wall motion abnormality. This study examined the differences in right ventricular performance between the two groups. There were 90 patients with a left ventricular ejection fraction less than 30% who had coronary angiography and multigated radionuclide angiography (MUGA). Of these, 69 had ischemic cardiomyopathy and 21 had primary cardiomyopathy. The left ventricular ejection fraction was similar; 22 +/- 6% in ischemic cardiomyopathy and 21 +/- 6% in primary cardiomyopathy. However, the right ventricular ejection fraction was higher in ischemic cardiomyopathy (38 +/- 16% versus 29 +/- 12%, p less than 0.01). There were 59 patients with right ventricular ejection fraction greater than or equal to 30%, of whom 50 patients (85%) had ischemic cardiomyopathy. The left ventricular and right ventricular volumes were determined by a count-based method. The right ventricular end-diastolic volume/left ventricular end-diastolic volume ratio was 0.57 in ischemic cardiomyopathy and 1.07 in primary cardiomyopathy (p less than 0.05). Thus assessment of right ventricular function may help differentiate primary from ischemic cardiomyopathy; a preserved right ventricular performance is highly suggestive of ischemic cardiomyopathy.

Cardiac Catheterization↗

Standardized patch infundibuloplasty for tetralogy of Fallot.

To preserve right ventricular function after intracardiac repair of tetralogy of Fallot, we developed a standardized patch infundibuloplasty with minimum ventriculotomy and myocardial resection. An experimental study suggested that short and medium-length patch infundibuloplasty, less than 50% of the right ventricular length, did not reduce the right ventricular function. A morphologic study showed that the ratio of the infundibular septal and the right ventricular lengths ranged from 0% to 34% with an average of 14% in 100 cases of tetralogy of Fallot. The optimal infundibuloplasty patch length was determined to be between 30% and 45% of the right ventricular length. The standard formulas for determining minimum (30%) and maximum (45%) patch infundibuloplasty lengths were as follows: 1.28 X body surface area (m2) + 1.10 cm (minimum) and 1.93 X body surface area (m2) + 1.65 cm (maximum). In clinical experience, a patch infundibuloplasty length shorter than 45% of the right ventricular length resulted in an intraoperative right ventricular to systemic arterial pressure ratio of 55% +/- 15% (n = 90).

Adolescent↗

Biventricular diastolic dysfunction in patients with autosomal-dominant polycystic kidney disease.

BACKGROUND: Left ventricular diastolic dysfunction has been shown in patients with autosomal-dominant polycystic kidney disease (ADPKD). However, there is no study evaluating right ventricular functions in these patients. METHODS: In the present study, diastolic functions of both ventricles in normotensive and hypertensive ADPKD patients with well-preserved renal function were investigated. Fifteen hypertensive and 16 normotensive patients with ADPKD with well-preserved renal function, 16 patients with essential hypertension, and 24 healthy subjects were included in the study. Conventional left and right ventricular echocardiographic measurements were performed in all subjects. Left and right ventricular functions were investigated both by myocardial performance index (MPI) [calculated by dividing the sum of isovolumic contraction time and isovolumic relaxation time (IVRT) by ejection time] and by tissue Doppler imaging (TDI). RESULTS: Left ventricular deceleration time and IVRT were significantly prolonged in hypertensive patients with ADPKD compared with patients with essential hypertension and even in normotensive patients with ADPKD compared with healthy subjects. Left and right MPIs were significantly higher in patients with ADPKD compared with healthy subjects, showing systolic and diastolic dysfunction. Moreover, by using TDI, the peak early diastolic mitral annular velocity (Em) to peak late diastolic mitral annular velocity (Am) ratio and the peak early diastolic tricuspid annular velocity (Et) to peak late diastolic tricuspid annular velocity (At) ratio were decreased in patients with ADPKD, suggesting biventricular diastolic dysfunction. CONCLUSION: Both hypertensive and normotensive patients with ADPKD show significant biventricular diastolic dysfunction, suggesting cardiac involvement very early in the course of ADPKD.

Adult↗

Reservoir and conduit function of right atrium: impact on right ventricular filling and cardiac output.

The purpose of this study was to investigate the relationship between right atrial (RA) reservoir and conduit function and to determine how hemodynamic changes influence this relationship and its impact on cardiac output. In 11 open-chest sheep, RA reservoir and conduit function were quantified as RA inflow with the tricuspid valve closed versus open, respectively. Conduit function was separated into early (before A wave) and late (after A wave) components. The effects of inotropic stimulation, partial pulmonary artery occlusion, and pericardiotomy were tested. At baseline with the pericardium intact, reservoir function accounted for 0.56 (SD 0.13) of RA inflow, early conduit for 0.29 (SD 0.07), and late conduit (during RA contraction) for 0.16 (SD 0.11). Inotropic stimulation decreased conduit function and increased reservoir function, but these effects did not reach statistical significance. With partial pulmonary artery occlusion, early conduit function fell to 0.20 (SD 0.11) (P < 0.04), and the conduit-to-reservoir ratio decreased by 41% (P < 0.03). Similarly, after pericardiotomy, early conduit function fell to 0.14 (SD 0.09) (P < 0.004), reservoir function increased to 0.72 (SD 0.08) (P < 0.04), and, consequently, the early conduit-to-reservoir ratio decreased by 63% (P < 0.006). Cardiac output was inversely related to the conduit-to-reservoir ratio (r = 0.39, P < 0.001). This study demonstrated that the right atrium adjusts its ability to act more as a reservoir than a conduit in a dynamic manner. The RA conduit-to-reservoir ratio was directly related to the right ventricular pressure-RA pressure gradient at the time of maximum RA volume, with increased ventricular pressures favoring conduit function, but it was inversely related to cardiac output, with an increase in the reservoir contribution favoring improved cardiac output.

Acute Disease↗

Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia.

STUDY OBJECTIVES: To determine (1) the prevalence of pulmonary hypertension and cardiac dysfunction in adult cystic fibrosis (CF) patients with severe lung disease, (2) the relationship between these cardiovascular abnormalities and hypoxemia, and (3) the impact of subclinical pulmonary hypertension on survival. DESIGN: Single-blind, cross-sectional study. SETTING: Ambulatory clinic of the Adult CF program at a tertiary-level hospital. PATIENTS: Clinically stable patients with severe lung disease (FEV1 < 40% of predicted normal value) who were not receiving supplemental oxygen. A second cohort of patients in stable condition with less severe lung disease (FEV1 40 to 65% predicted) was also recruited to enable multivariate analysis for the determinants of pulmonary hypertension. MEASUREMENTS AND RESULTS: Eighteen patients with severe lung disease (FEV1 28 +/- 7% of predicted normal value) were initially studied. Each patient had overnight polysomnography, pulmonary function tests, and Doppler echocardiography. Arterial oxygen saturation (SaO2) was reduced during wakefulness (87.1 +/- 6.1%) and fell during sleep (84.0 +/- 6.6%) while transcutaneous PCO2 was normal during wakefulness (41.1 +/- 6.9 mm Hg) and increased during sleep (46.6 +/- 4.7 mm Hg). Left ventricular size, systolic function, and diastolic function were normal except in one patient who had had a previous silent myocardial infarction due to coronary artery disease. Qualitative assessment of right ventricular function was normal in all patients. Pulmonary artery systolic pressure (PASP) was increased (> 35 mm Hg) in seven patients without clinical evidence of cor pulmonale. Regression analysis was performed by combining these data with data from an additional 15 CF patients with moderately severe lung disease (FEV1 56.3 +/- 8.9% predicted normal) who were recruited to a modified study protocol that included overnight oximetry, pulmonary function tests, and Doppler echocardiography. None of these patients had evidence of hypoxemia and only three had mild elevation of PASP (36, 37, and 39 mm Hg). Linear regression analysis revealed that PASP was significantly correlated with FEV1 (r = -0.44; p = 0.013), and SaO2 during wakefulness (r =-0.60; p = 0.0003), during sleep (r = -0.56; p = 0.0008), and after 6 min of exercise (r = -0.75; p < 0.0001). Multivariate analysis revealed that awake SaO2 was a significantly better predictor of PASP than FEV1 (p = 0.0104). Clinical follow-up of the original cohort for up to 5 years revealed that mortality was significantly higher in those with pulmonary hypertension than those without pulmonary hypertension (p = 0.0129). CONCLUSIONS: In adult CF patients with severe stable lung disease, left and right ventricular function is well maintained in the absence of significant coronary artery disease; pulmonary hypertension develops in a significant proportion of patients and is strongly correlated with oxygen status, independent of lung function; and subclinical pulmonary hypertension is associated with an increased mortality.

Adult↗

Quantification of right and left ventricular function by cardiovascular magnetic resonance.

Cardiac dysfunction is a major cause of cardiovascular morbidity and mortality. Accurate and reproducible assessment of cardiac function is essential for the diagnosis, the assessment of prognosis and evaluation of a patient's response to therapy. Cardiovascular Magnetic Resonance (CMR) provides a measure of global and regional function that is not only accurate and reproducible but is noninvasive, free of ionising radiation, and independent of the geometric assumptions and acoustic windows that limit echocardiography. With the advent of faster scanners, automated analysis, increasing availability and reducing costs, CMR is fast becoming a clinically tenable reference standard for the measurement of cardiac function.

Echocardiography↗

Echocardiographic analysis of the subtypes of right ventricular restrictive physiology in surgically treated patients with tetralogy of Fallot.

BACKGROUND: Our study focuses on echocardiographic assessment of the right ventricular diastolic function and adaptive right ventricular response to volume overload resulting from pulmonary and tricuspid regurgitation in surgically treated patients with tetralogy of Fallot. METHOD AND RESULTS: We included 60 patients subsequent to surgical correction of tetralogy of Fallot, dividing them into two groups - a group of 18 patients with restrictive physiology, having antegrade flow to the pulmonary arteries greater than 30 centimetres per second in late diastole in five consecutive beats, and a group of 42 patients with non-restrictive physiology. Based on the cardiothoracic ratio, being more or equal to, or less than 0.55, we further divided those with restrictive physiology into a group of 14 patients deemed to have primary restriction, and the other 4 patients considered to have secondary or acquired restriction. Those with non-restrictive physiology were divided into groups of 16 patients with a small heart, and 26 patients with a large heart. A fraction of the venous retrograde diastolic flow in the hepatic vein greater or equal to 30 centimetres is important for distinguishing between the subgroup with primary restriction and the other subgroups. In the four patients with secondary restriction, anterograde diastolic flow in the pulmonary artery greater than 30 centimetres per second was recorded after the average period of follow-up of 2.4 years. The mean value of the pulmonary regurgitant jet pressure half-time was higher in the subgroup with the secondary restriction in comparison to the nonrestrictive subgroup with large hearts at 152 milliseconds with standard deviation of 36 milliseconds versus 85 milliseconds with standard deviation of 11 milliseconds, p less than 0.05. This was significantly lower in comparison to those with primary restriction, where the value was 238 milliseconds, with standard deviation of 42 milliseconds, p less than 0.02. CONCLUSION: Echocardiographic analysis offers great possibilities for assessment of right ventricular diastolic function, identifying in particular those with restrictive physiology, its interrelation with pulmonary and tricuspid regurgitation, as well as timing and selection of patients for re-intervention.

Child↗

Ventricular function in myocardial contusion: a preliminary study.

During a consecutive 17 month period, 15 trauma patients were diagnosed as having a myocardial contusion on the basis of abnormal ECG in 14 patients, elevated creatinine phosphokinase (CPK) in 13 patients, and elevated CPK-Muscle Brain (CPK-MB) isoenzyme determination in 11 patients. Using these screening modalities, the incidence of myocardial contusion in patients with blunt chest trauma increased from 7% when viewed retrospectively to 15% when viewed prospectively. Five patients had determinations of cardiac index of which 4 were less than 2.9 L/min . M2. An additional 9 patients underwent a standard fluid challenge of 500 ml of 5% plasma protein faction infused over 30 min allowing construction of a Starling myocardial performance curve. Of these patients, 6 had biventricular dysfunction, 1 had isolated abnormal left ventricular function, and 2 had isolated abnormal right ventricular function in the absence of chronic obstructive pulmonary disease or preexisting heart disease. Multiple gated acquisition scans (MUGA) were abnormal in 6 patients and normal in 4 patients. Of the latter group, 3 had biventricular dysfunction and 1 had depressed cardiac index. Morbidity and mortality for myocardial contusion occurred in 40% (6 of 15) of patients in this series. Direct hemodynamic measurement with construction of a Starling curve was useful in monitoring the degree of impairment and subsequent recovery of myocardial function. This information was important in delaying semiurgent operations or in determining the best alternative of otherwise equally acceptable methods of patient management.

Adolescent↗

Determinants of recurrent or residual functional tricuspid regurgitation after tricuspid annuloplasty.

BACKGROUND: The durability of tricuspid valve (TV) repair by annuloplasty is limited. Identification of mechanisms of recurrent or residual tricuspid regurgitation (TR) after annuloplasty is necessary to improve results of TV repair. The purpose of this study was to investigate echocardiographic determinants of mid-term outcome after TV annuloplasty. METHODS AND RESULTS: This study consisted of 39 patients with functional TR who had echocardiography preoperatively, early postoperatively (5+/-2 days), and >1 year (20+/-6 months) after TV annuloplasty. Detailed echocardiographic measurements were performed, including TR severity, TV annular dimension, TV leaflet displacement, left ventricular (LV) function, and right ventricular (RV) function and pressures. Preoperative leaflet tethering height and area predicted early and mid-term outcome of annuloplasty. Early postoperative LV ejection fraction and TR severity influenced degree of TR >1 year after surgery. In addition, increased RV pressure was related to worse TR during late follow-up. CONCLUSIONS: Although TV tethering is an important determinant of recurrent or residual TR, LV and RV function and pressures impact repair durability. These factors identify patients at risk for repair failure; such individuals require development of additional surgical strategies to improve results of tricuspid valve repair and close surveillance after surgery.

Aged↗

LVAS pump performance following initiation of left ventricular assistance.

Prevention of disturbed flow (e.g., flow stasis) and consequent thrombosis in heart pumps is based upon design characteristics determined during laboratory bench tests. These tests employ optimal filling and emptying characteristics, such as the full-fill to complete empty mode in the Novacor left ventricular assist system. Filling characteristics of the Novacor LVAS were examined during the first 48 hours after implantation in 14 patients. Fill volume of the pump was reduced in pathologic states, such as cardiac tamponade, and following the initiation of right ventricular mechanical circulatory support. In addition, multiple regression analysis revealed that right ventricular function measured by the amount of inotropic support required, the right ventricular ejection fraction, and the total pulmonary resistance, significantly predicted left ventricular assist pump fill volume during the first 48 hours of support. Flow visualization simulating these clinical conditions of incomplete filling suggest inadequate valve washing, particularly around the inlet valve and its conduit, which may predispose to thrombus formation.

Cardiac Output↗

Additional predictive value of both left and right ventricular ejection fractions on long-term survival in idiopathic dilated cardiomyopathy.

BACKGROUND: In idiopathic dilated cardiomyopathy, long-term outcome is poor and left ventricular ejection fraction is a major powerful predictor of survival. However, right ventricular function might also play an important role in the long-term prognosis of this disease. AIM: The aim of this study was to determine the role of right ventricular parameters, mainly right ventricular ejection fraction, on survival in idiopathic cardiomyopathy. METHODS: We prospectively assessed long-term follow-up and predictors of survival in 62 consecutive patients referred from 1990 to 1992 for evaluation of idiopathic dilated cardiomyopathy, including haemodynamic evaluation, thermodilution right ventricular ejection fraction and volume measurements. RESULTS: At the time of catheterization, dyspnoea class III or IV was present in 60% of the patients, atrial fibrillation in 19% and complete left bundle branch block in 35%. Left ventricular ejection fraction was 30 +/- 10% and right ventricular ejection fraction was 30 +/- 16%. During follow-up (2.2 +/- 1.3 years), 15 patients (24%) had heart transplantation and nine (14%) died before cardiac transplantation. Cumulative survival rate without heart transplantation was 74% and 56% at 1 and 4 years, respectively. In univariate analysis, survival was related to: dyspnoea class I or II (P < 0.04), absence of complete left bundle branch block (P < 0.05), administration of lower doses of furosemide (P < 0.01), high left ventricular ejection fraction (P < 0.001), low pulmonary artery pressure (P < 0.002), high cardiac index (P < 0.006), and low right ventricular volumes (P < 0.001). Multivariate analysis showed only two independent predictors of survival: left ventricular ejection fraction (P < 0.001) and right ventricular ejection fraction (P < 0.004). CONCLUSION: In addition to left ventricular ejection fraction, right ventricular ejection fraction appears to be a complementary predictor of survival in idiopathic dilated cardiomyopathy, suggesting the importance of assessing right ventricular function in this disease.

Adult↗

The pericardial membrane pulmonary monocusp: surgical technique and early results.

Long-term pulmonary insufficiency resulting from simple transannular patching of the right ventricle outflow tract will ultimately lead to deterioration in right ventricular function. Previously, monocusps constructed from xenografts, homografts, fascia lata, and autologous pulmonary artery wall have been utilized to minimize pulmonary regurgitation and its deleterious effect on right ventricular function. However, these tend to degenerate in the long term, necessitating reoperation. To circumvent this problem we have used a monocusp constructed from 0.1-mm polytetrafluorethylene (PTFE, pericardial membrane) clinically demonstrated to be resistant to issue ingrowth and degeneration. Seven children (5 tetralogy of Fallot, 2 pulmonary stenosis) who required division of a small pulmonary annulus underwent monocusp construction with 0.1-mm PTFE. Three patients had previous corrective surgery. One of these patients had a bovine pericardial monocusp placed 8 years previously, which degenerated. Of the remaining two patients, one had a pulmonary valvotomy as a neonate, the other repair of tetralogy of Fallot with a transannular patch. At a mean (+/- standard deviation) follow up to 17+/-5.8 months all patients are alive and are New York Heart Association (NYHA) Class I. Echocardiography demonstrates mild pulmonary insufficiency (PI) in 2 patients, mild to moderate PI in 4, and moderate to severe PI in 1. The presence of a pericardial membrane monocusp in the pulmonary position may, in the long term, prevent the deleterious effects of transannular patching on right ventricular dysfunction and be more resistant to degenerative changes characteristic of monocusps constructed of native pericardium or allogeneic tissue.

Adult↗

Pedicled pericardium for repair of right ventricular outflow tract and pulmonary arterial stenoses in tetralogy of Fallot: a six-year experience.

From June 1988 through June 1994, 20 children with symptomatic tetralogy of Fallot, associated with hypoplastic or stenotic pulmonary arteries in 19 cases, were operated on in our institution. Mean age at operation was 49.5 +/- 43 months (ranging from 10 months to 12.5 years). Mean weight was 13.5 +/- 6.5 kg (range 6.2 to 30 kg) and mean body surface area was 0.6 +/- 0.2 m2. Mean preoperative hematocrit value was 47.6% +/- 11.1%, and mean preoperative arterial oxygen saturation ratio was 75.7 +/- 9.5. Six patients (30%) had prior systemic-pulmonary arterial shunts. Pulmonary arterial stenoses were congenital or iatrogenic (or both) and were located principally on the left pulmonary artery (63%), on the pulmonary bifurcation (15%), or separately on the left and right pulmonary arteries (21%). In all children the pulmonary arterial tree was repaired with the patient's own pedicled pericardium. Mean follow-up is 36 +/- 25.2 months (range 1 to 71 months). Hospital mortality rate was 0%. There was one late death, and two children were lost to long-term follow-up. No child required reoperation. Seventeen children returned for evaluation, consisting of physical examination, electrocardiogram, chest roentgenogram, and Doppler echocardiogram, and 11 underwent late catheterization or magnetic resonance imaging of the chest to evaluate the pulmonary arterial repair. All children were in New York Heart Association class I or II. Right ventricular function was normal by echocardiography in 100% with a mean right ventricular systolic pressure of 39.37 +/- 8.4 mm Hg. Mild to moderate pulmonary regurgitation was present in the majority of patients. The results of pulmonary arterial repair were good in 100%. There was no residual stenosis, and we observed uniform enlargement of the repaired pulmonary arteries over a 5-month to 6-year follow-up period. These results are of particular interest inasmuch as other materials previously used for repair of pulmonary arteries do not grow and may even shrink, resulting in residual stenosis.

Body Surface Area↗