Prenatal diagnosis of cardiac defects: accuracy and benefit.

OBJECTIVE: The prenatal diagnosis of cardiac defects can potentially reduce postnatal morbidity and mortality. We wanted to evaluate prenatal cardiac diagnosis accuracy in a population referred for echocardiography. METHODS: Single centre retrospective study of echocardiography referrals between April 1999 and December 2008. We compared the prenatal and postnatal cardiac diagnoses, the modified Aristotle and Wald scores. The final diagnosis Wald score was used to evaluate benefit. RESULTS: Six hundred fetuses were included. Diagnoses included: normal heart (312, 52%); congenital heart defect (CHD) (231, 38.5%); primary arrhythmia (39, 6.5%); or cardiomyopathy, myocarditis or cardiac tumor (18, 3%). The prenatal and postnatal Aristotle and Wald scores correlated in 81% and 86%, respectively, each with significant differences in 22 cases. Four significant CHDs were misdiagnosed, the surgical prediction was incorrect in 7 and 13 false positive diagnoses of aortic coarctation were made. In 76% (455/600) fetuses prenatal diagnosis was considered beneficial. The average CHD Aristotle score was 9.5 ± 5.0. In babies with CHDs and normal karyotype the score was either 6.5 ± 5.0, 12.9 ± 3.1 or 13.2 ± 2.9, in survivors, cases of postnatal demise and cases of pregnancy termination, respectively. CONCLUSION: Prenatal diagnosis was accurate and the counselling appropriate in most cases; however, a few errors were made. The diagnosis of aortic coarctation remains challenging.

Cardiac function in trisomy 21 fetuses.

OBJECTIVES: Trisomy 21 is associated with an increased nuchal translucency thickness (NT), abnormal ductus venosus (DV) flow at 11-14 weeks' gestation and congenital heart defects (CHD), and cardiac dysfunction has been hypothesized as the link between them. We therefore aimed to investigate whether cardiac function is altered in trisomy 21 fetuses. METHODS: Between December 2003 and June 2009, we performed echocardiography on 46 trisomy 21 fetuses (28 with structurally normal heart and 18 with CHD) and on 191 chromosomally/phenotypically normal fetuses with a confirmed normal heart (87 with normal NT and 104 with NT ≥ 95(th) percentile), between 11 and 35 weeks' gestation. Measurements included: E- and A-wave peak velocity, E/A velocity ratio and E/time velocity integral (TVI) ratio over atrioventricular valves; myocardial performance index (MPI); semilunar valve peak velocity and acceleration time; stroke volume (SV); cardiac output; and DV pulsatility index for veins (PIV) at 11-14 weeks' gestation. Data were categorized into three different age groups for analysis (11 to 13 + 6, 14 to 21 + 6 and 22 to 35 weeks' gestation). RESULTS: The tricuspid valve (TV) A-wave velocity and aortic valve peak velocity were significantly reduced in trisomy 21 compared with normal fetuses. Other highly significant differences found in trisomy 21 fetuses at 11-14 weeks' were increased TV-E/A ratio and DV-PIV, and decreased pulmonary valve peak velocity. We also observed evidence of left ventricular (LV) systolic dysfunction, reduced SV and increased MPI. After 14 weeks' gestation, the mitral valve A-wave peak velocity and E/TVI ratio were significantly reduced in the trisomy 21 fetuses with normal hearts compared with the controls with increased NT. CONCLUSIONS: In comparison with controls with normal or increased NT, cardiac function in trisomy 21 fetuses is abnormal irrespective of the presence of CHD. Evidence for cardiac loading (increased preload and afterload) and LV systolic (in the first trimester) and later diastolic dysfunction was observed.

Fetal cardiac function between 11 and 35 weeks' gestation and nuchal translucency thickness.

OBJECTIVES: The pathophysiological background of an increased nuchal translucency (NT) is still poorly understood. Cardiac dysfunction has been proposed as a cause. The aim of this study was to determine if, in fetuses with normal hearts, the NT thickness is related to cardiac function throughout gestation. METHODS: The NT was measured in 191 karyotypically/phenotypically normal fetuses with structurally normal hearts and was increased (≥ 95(th) centile) in 104. All fetuses had been referred for fetal echocardiography and were prospectively included between October 1 2003 and April 1 2009. Three-hundred and ten echocardiograms were performed between 11 and 35 weeks' gestation. The E- and A-wave velocity, E/A velocity ratio, E/time velocity integral (TVI) ratio over the atrioventricular (AV) valves, myocardial performance index, acceleration time (AT) and peak velocity over the semilunar valves, stroke volume (SV) and cardiac output (CO) as well as the ductus venosus pulsatility index for veins at 11-14 weeks' gestation (DV-PIV), were measured. A multilevel analysis was performed using the NT multiples of the median (MoM) as a continuous variable. RESULTS: AV-E- and A-wave velocities, E/A velocity ratios, semilunar valve peak velocity, SV, CO and aortic valve (AoV) AT increased significantly with advancing gestation. At 11-14 weeks' gestation, the AoV-AT, tricuspid valve (TV)-E/A, TV-E/TVI ratios and DV-PIV increased, and the pulmonary valve (PV) AT decreased, with increasing NT-MoMs. After midgestation, the PV-AT increased and the AoV-AT, TV-E/A and TV-E/TVI ratios decreased with increasing NT-MoMs. CONCLUSIONS: NT thickness is related to right ventricular diastolic function and semilunar valve AT. Our findings suggest improved first-trimester, but later reduced, right ventricular relaxation and discordant ventricular afterload in fetuses with an increased NT.

Is fetal cardiac function gender dependent?

INTRODUCTION: An increased nuchal translucency (NT) is more common in males. A delayed diastolic cardiac function maturation has been proposed to explain this and the reported gender-related differences in ductus venosus (DV) flow. OBJECTIVE: To investigate gender-related differences in fetal cardiac function. METHODS: One hundred and ninety karyotypically/phenotypically normal fetuses with structurally normal hearts and known NT measurement, (104 > 95th percentile), were prospectively included between 1 October 2003 and 1 April 2009. They had been referred for fetal echocardiography. Three hundred and nine echocardiograms were performed between 11 and 35 weeks' gestation. The atrioventricular valve E- and A-wave peak velocity, E/A-velocity ratio and E/TVI ratio, myocardial performance index, semilunar valves acceleration time (AT) and peak velocity, stroke volume and cardiac output as well as DV pulsatility index for veins at 11-14 weeks' gestation, were measured. A multilevel analysis was performed using the NT (multiples of the median) as a continuous variable. RESULTS: The male : female ratio was 1.56:1. The tricuspid valve E/TVI was significantly higher and pulmonary valve AT significantly lower in females compared to males. No other significant differences in cardiac function were found. CONCLUSIONS: Our findings suggest better right ventricular (RV) relaxation and increased RV afterload in female fetuses, independent of NT thickness, between 11 and 35 weeks' gestation.

Audit of 10 years of referrals for fetal echocardiography.

OBJECTIVES: To evaluate trends over time, indications, diagnoses, noncardiac defects and outcome of fetuses referred for tertiary level echocardiography. METHODS: Retrospective study of fetal echocardiograms performed between April 1999 and 2009. RESULTS: Of the 623 fetuses included, 301 (48%) had cardiac pathology. Congenital heart defects (CHDs) were found in 243/301 (81%), mostly in the severe spectrum. Of the fetuses with CHDs, 26% (63/243) had chromosomal anomalies. The chromosomally normal fetuses with CHDs had a mortality rate of 43% (77/180) and 23% (41/180) had extra-cardiac anomalies. The termination of pregnancy (TOP) rate for all cardiac pathology was 24.9% (75/301) and for CHDs 29.6% (72/243). The TOP rates for CHDs diagnosed before 19 and 24 weeks gestation were 61% (28/46) and 44% (68/155), respectively. An increase in referrals followed the introduction of a national screening program, (nuchal translucency (NT) and routine structural ultrasound screening). The main referral indication was an increased NT (>95th percentile; 32% of cases). CHDs were found in 81/239 (34%) fetuses with an increased NT. CONCLUSIONS: Referral indications for fetal echocardiography were appropriate (almost 50% had cardiac pathology). The mortality was high. Fetal outcome and TOP decisions correlated with CHD severity and presence of noncardiac defects. An increased NT is a strong marker for CHDs.

Echocardiographic evaluation of fetal cardiac function: clinical and anatomical correlations in two cases of endocardial fibroelastosis.

BACKGROUND: Two fetuses with endocardial fibroelastosis, one with critical aortic stenosis and one with high-output cardiac failure due to chorioangiomatosis, are presented to evaluate the correlation between Doppler echocardiographic findings, the fetal clinical condition and the anatomical substrate found at postmortem. METHODS: Doppler measurements of cardiac function (systolic, diastolic and global) and a cardiovascular score incorporating five parameters of fetal well-being were recorded. RESULTS: In the fetus with critical aortic stenosis, the cardiovascular score was diminished, there was no hydrops, the systolic and global cardiac function indices were within normal limits but the diastolic function indices were abnormal. The fetus with high-output cardiac failure was hydropic, the cardiovascular score was diminished and abnormal Doppler indices of systolic, diastolic and global cardiac function were found. In both fetuses, abnormalities in the measured Doppler parameters were found consistent with clinical cardiac dysfunction and the postmortem findings. CONCLUSION: Recognition of abnormal diastolic function Doppler indices may assist in earlier identification of fetal cardiac compromise.

The nuchal translucency and the fetal heart: a literature review.

In this overview the current knowledge of the relationship between an increased nuchal translucency (NT) measurement and fetal heart structure and function in chromosomally normal fetuses is reviewed. Relevant pathophysiological theories behind the increased NT are discussed. Fetuses with an increased NT have an increased risk for congenital heart disease (CHD) with no particular bias for one form of CHD over another. This risk increases with increasing NT measurement. Although the NT measurement is only a modestly effective screening tool for all CHD when used alone, it may indeed be effective in identifying specific CHD "likely to benefit" from prenatal diagnosis. The combination of an increased NT, tricuspid regurgitation and an abnormal ductus venosus (DV) Doppler flow profile, is a strong marker for CHD. A fetal echocardiogram should be performed at 20 weeks' gestation in fetuses with an NT > or = 95th percentile but < 99th percentile. When the NT measurement is > or = 99th percentile, or when tricuspid regurgitation and/or an abnormal DV flow pattern is found along with the increased NT, an earlier echocardiogram is indicated, followed by a repeat scan at around 20 weeks' gestation. The resultant increased demand for early fetal echocardiography and sonographers with this special expertise needs to be planned and provided for.

[Percutaneous implantation of a pulmonary valve in 3 children with surgically corrected cardiac anomalies]. / Percutane implantatie van een pulmonalisklep bij 3 kinderen met chirurgisch gecorrigeerde hartafwijkingen.

An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implantation of a Melody pulmonary valve prosthesis to replace a stenotic and insufficient homograft in the pulmonary artery. Preoperatively, 2 of the children suffered from fatigue and dyspnoea on exertion The homografts had been implanted between the ages of 1-2, to establish surgical continuity between the right ventricle and the pulmonary artery. The anomalies were tetralogy of Fallot, pulmonary atresia with intact ventricular septum and pulmonary atresia with a ventricular septum defect. Percutaneous pulmonary valve replacement was successful in all 3 patients. After implantation, right ventricular pressure decreased to 30% of systemic pressure and regurgitation was not observed. All patients were discharged in a good condition on the day after the implantation. Percutaneous pulmonary valve replacement is a promising technique with good short-term results. In selected patients this percutaneous technique can substitute or postpone the surgical replacement ofa stenotic or insufficient homograft.

Percutaneous implantation of a pulmonary valve: an illustrative case.

Surgical reconstruction of the right ventricular outflow tract (RVOT) with valved conduits in infants and children with congenital heart disease leads to re-intervention in later life as the ensuing pulmonary regurgitation and stenosis of the degenerating conduit impacts negatively on right ventricular function. Percutaneous pulmonary valve implantation (PPVI) provides a safe alternative to early surgical re-intervention in these patients. We describe this procedure as performed on an 11- year-old boy. Difficulty may be experienced crossing the RVOT prior to PPVI. We describe several techniques that may be used to encourage the distal movement of the delivery system through the RVOT. (Neth Heart J 2007;15:27-30.).

[Favourable results with surgical treatment in 43 children with hypoplastic left-heart syndrome or similar disorders, 1999-2005]. / Gunstige resultaten van chirurgische behandeling van 43 kinderen met het hypoplastisch linkerhartsyndroom of een equivalente afwijking, 1999/'05.

OBJECTIVE: To describe the results of surgical treatment of hypoplastic left-heart syndrome (HLHS) and HLHS-like disorders in the Amsterdam-Leiden Centre for Congenital Heart Disease, the Netherlands. DESIGN: Retrospective, descriptive. METHOD: Data were collected on 43 neonates with HLHS or similar disorders who underwent surgical treatment between December 1999 and December 2005. HLHS was present in 37 patients and 6 had disorders similar to HLHS (unbalanced atrioventricular septal defect, truncus arteriosus with hypoplastic left ventricle, double inlet left ventricle). Surgery was performed in 3 steps: Norwood operation shortly after birth (n = 43), bidirectional cavopulmonary anastomosis a few months later (n = 30) and total cavopulmonary connection at the age of 2-3 years (n = 10). During the Norwood operation, the first 21 patients received a modified Blalock shunt (between the right brachiocephalic artery and pulmonary artery), whereas the following 22 patients received a Sano shunt (between the right ventricle and pulmonary artery). RESULTS: Of the 43 patients, 11 died: 7 within 30 days of the first operation, 2 between the first and second operation, and 2 between the second and third operation. Actuarial survival for the entire group is 74% (32/43). The mortality rate was lower with the Sano shunt (9%; 2/22) than with the modified Blalock shunt (43%; 9/21). Catheter interventions were necessary in 10 patients: 6 had balloon dilatation of the distal aortic arch and 4 had balloon dilatation/stent placement for narrowed pulmonary arteries. With a median follow-up of 22 months (range: 1-75), 2 patients had marked neurological side effects. All 32 surviving patients were in good clinical condition.

Anthracycline-induced clinical heart failure in a cohort of 607 children: long-term follow-up study.

PURPOSE: To determine the early and late cumulative incidence of anthracycline-induced clinical heart failure (A-CHF) after anthracycline therapy in childhood and to identify associated risk factors. PATIENTS AND METHODS: The cumulative incidence of A-CHF and the risk factors of A-CHF were assessed in a cohort of 607 children who had been treated with anthracyclines between 1976 and 1996. For 96% of the cohort, we obtained the clinical status up to at least January 1997. The mean follow-up time was 6.3 years. RESULTS: The cumulative incidence of A-CHF was 2.8%, after a mean follow-up time of 6.3 years and a mean cumulative dose of anthracyclines of 301 mg/m(2). A cumulative dose of anthracycline higher than 300 mg/m(2) was associated with an increased risk of A-CHF (relative risk, 11.8; 95% confidence interval, 1.6 to 59.5) compared with a cumulative dose lower than 300 mg/m(2). The estimated risk of A-CHF increased with time after the start of anthracycline chemotherapy to 2% after 2 years and 5% after 15 years. CONCLUSION: Up to 5% of patients will develop A-CHF 15 years after treatment, and patients treated with a cumulative dose of anthracyclines higher than 300 mg/m(2) are at highest risk for A-CHF. This is thus a considerable and serious problem among these young patients. The findings reinforce the need for strategies for early detection of patients at risk for A-CHF and for the evaluation of other chemotherapeutic possibilities or cardioprotective agents in relation to the survival.

Indium-111-antimyosin scintigraphy in the early detection of heart damage after anthracycline therapy in children.

PURPOSE: To determine the value of indium-111-antimyosin ((111)In-AM) scintigraphy in the early detection of myocardial damage in children treated with doxorubicin. PATIENTS AND METHODS: Twelve planar scintigrams were made of eight patients (seven children and one young adult; mean age, 12 years). Three scans were obtained before doxorubicin therapy in three patients, and nine scans were obtained during doxorubicin therapy in seven patients. The heart-to-lung ratio (HLR) was calculated. Left ventricular function was assessed by echocardiography before and during therapy by measuring the fractional shortening (FS). RESULTS: The HLR of the three baseline scans was below 1.5, within the normal range for adults. Six of the seven patients whose scans were obtained during chemotherapy had abnormal HLR values (> 1.5). One patient had severe myocyte damage and showed an early increase in the HLR (2.3) after a cumulative doxorubicin dose of 150 mg/m(2). The FS measured by echocardiography was normal throughout therapy, and the final cumulative dose of doxorubicin was 450 mg/m(2). This patient developed fatal clinical heart failure 3 months after completion of chemotherapy. In one patient, who was pretreated with the cardioprotective agent dexrazoxane, the HLR remained within normal limits during therapy. CONCLUSION: (111)In-AM scintigraphy seems to be suitable to detect early myocardial damage after a cumulative doxorubicin dose of 150 mg/m(2 )in children and may be useful for identifying children who are at increased risk of developing cardiac sequelae.

Right ventricular diastolic function in children with pulmonary regurgitation after repair of tetralogy of Fallot: volumetric evaluation by magnetic resonance velocity mapping.

OBJECTIVES: We sought to assess right ventricular diastolic function in young patients with corrected tetralogy of Fallot and pulmonary regurgitation. BACKGROUND: Pulmonary regurgitation is an important problem in repair of tetralogy of Fallot. Its effects on right ventricular diastolic function in children are unknown. METHODS: Nineteen children with repair of tetralogy of Fallot (mean age [+/- SD] 12 +/- 3 years, mean age at operation 1.5 +/- 1) and 12 healthy children were studied. Summation of magnetic resonance velocity mapping pulmonary and tricuspid volume flow curves provided right ventricular time-volume curves. Ventricular size was assessed with tomographic magnetic resonance imaging (MRI). Graded exercise testing was performed. RESULTS: Systematic and random differences (mean +/- SD) of velocity mapping and Doppler tricuspid time to peak velocities (peak E: 1 +/- 26 ms, r = 0.43; peak A: 2 +/- 11 ms, r = 0.76), E/A ratio (0.04 +/- 0.5, r = 0.63) and duration of pulmonary regurgitation (20 +/- 35 ms, r = 0.74) were satisfactory. In 6 patients (group I), late diastolic forward pulmonary artery flow was absent; in 13 patients (group II), this flow contributed 1% to 14% to right ventricular stroke volume. Significant differences were increased deceleration time (315 +/- 91 vs. 168 +/- 28 ms, p < 0.001), decreased filling fraction (44 +/- 11 vs. 55 +/- 16%, p = 0.02) and increased peak early filling rate (378 +/- 124 vs. 286 +/- 112 ml/s, p = 0.018) between control subjects and group I, and increased deceleration time (230 +/- 40, p = 0.03) between control subjects and group II. Pulmonary regurgitation, ventricular size and ejection fraction did not differ significantly between patient groups. Exercise function was diminished with restrictive right ventricular physiology (p < 0.001, group II vs. control subjects). CONCLUSIONS: Impaired relaxation and restriction to filling affect right ventricular function in children with repair of tetralogy of Fallot and pulmonary regurgitation. Restrictive right ventricular physiology is associated with decreased exercise function.

Postoperative pulmonary flow dynamics after Fontan surgery: assessment with nuclear magnetic resonance velocity mapping.

OBJECTIVES: This study was performed to assess the value of nuclear magnetic resonance (NMR) velocity mapping for the measurement of pulmonary blood flow after Fontan surgery. BACKGROUND: Echocardiographic studies of pulmonary flow after Fontan surgery are not always satisfactory. The newly developed technique of NMR velocity mapping may contribute to the elucidation of the Fontan circulation. METHODS: At frequent intervals during the cardiac cycle, forward and backward flow volumes in the pulmonary arteries of nine volunteers were measured, summed and compared with right ventricular stroke volume to validate the velocity mapping technique. In 14 patients after Fontan surgery, assessment of pulmonary flow volumes enabled the evaluation of atriopulmonary and atrioventricular (AV) Fontan connections. The findings were correlated with precordial echocardiography. RESULTS: Validation of the NMR technique, obtained from volunteer experiments, showed a high correlation (r = 0.97) between right ventricular stroke volume and volumetric pulmonary stroke flow. In all patients with an atriopulmonary Fontan connection (n = 8), forward flow in the pulmonary artery was biphasic, similar to normal venous flow. Monophasic systolic pulmonary flow curves indicating right ventricle-dependent pulmonary blood flow were found in three of six patients with an AV Fontan connection. In the remaining three patients, the pulmonary flow pattern did not reflect right ventricular contraction. Measurement of flow velocity alone may give a false impression of forward flow and thus of right ventricular contribution. Pulmonary regurgitation was demonstrated in six of eight patients with an atriopulmonary connection. CONCLUSIONS: Nuclear magnetic resonance velocity mapping provides accurate and valuable information on pulmonary flow volume and velocity after Fontan surgery. The success of AV Fontan surgery can be deduced from the presence of a monophasic systolic pulmonary flow pattern as demonstrated by NMR velocity mapping. With NMR flow volume analysis, substantial pulmonary regurgitation occurring after atriopulmonary Fontan surgery can be measured.

[Complete atrioventricular septal defect in children with Down's syndrome: good results of surgical correction at younger and younger ages]. / Compleet atrioventriculair septumdefect bij kinderen met het syndroom van Down: goede resultaten van chirurgische correctie op steeds jongere leeftijd.

OBJECTIVE: To evaluate the results of cardiosurgical treatment of children with Down's syndrome and a complete atrioventricular septal defect (cAVSD). DESIGN: Retrospective. METHOD: Data were collected from the records of all patients with Down's syndrome who had been subjected to primary corrective surgery for cAVSD in the period 1980-2003 in Leiden, The Netherlands. Exclusion criteria were: concomitant tetralogy of Fallot or interruption of the aortic arch and palliative banding of the pulmonary artery. Children with an abnormal shape of the ventricle making it impossible to correct biventricularly were not considered in the study. Data on mortality >30 days after the operation and on repeated surgery were included in the analysis if the duration of follow-up was at least 5 years. RESULTS: The group comprised 148 children: 75 girls and 73 boys. The median age at time of surgery was 20 weeks (range: 6 weeks to 3.7 years) and showed a statistically significant decrease during the period under investigation. Of 4 children lost to clinical follow-up actual data could be obtained. The median duration offollow-up was 6 years and 7 months (range: 38 days to 23 years and 11 months). 28 children (19%) died. The mortality within 30 days after the operation decreased from 0-38% in the period 1980-1989 via 0-30% in the period 1990-1999 to 0% in 2000-2003. The percentage ofreoperations related to the correction ofcAVSD (14%; 14/98) did not seem to increase. Of the correction-related reoperations, 14% (2/14) were followed by a second reoperation. Conclusion. In the period 1980-2003, children with Down's syndrome and a cAVSD were corrected surgically at a younger and younger age. The early mortality decreased to 0% in the years 2000-2003. Echocardiography in the first weeks of life for all children with Down's syndrome makes it possible to diagnose those with a cAVSD early enough for surgical repair.

Normal development of the pulmonary veins in human embryos and formulation of a morphogenetic concept for sinus venosus defects.

A sinus venosus defect is a form of interatrial communication associated with abnormal drainage of the right pulmonary veins. Its morphogenesis still remains unclear. We therefore studied the normal development of pulmonary veins in human embryos in relation to the sinus venosus and the dorsal mesocardium using graphic reconstructions and HNK-1 immunohistochemistry. Twenty embryos, ranging from 4 to 7 weeks' gestation, were examined. At 4 weeks, the orifice of the nonlumenized common pulmonary vein is visible as an endothelial invagination within the sinus venosus segment. Development of the muscular septum primum and the ventral proliferation of extracardiac mesenchyme from the dorsal mesocardium positions the common pulmonary vein (CPV) eventually into the left atrium. The right wall of the CPV contributes to the posterior part of the atrial septum and is continuous with the dorsal sinuatrial fold (the future left venous valve). With the use of HNK-1 antigen expression as a marker for sinus venosus myocardium, this common wall between the right-sided sinus venosus and the CPV is demonstrated, and at 7 weeks the proximal part of the right upper pulmonary vein also becomes part of this common wall. This study demonstrates that the CPV develops within the sinus venosus segment and that later a common myocardial wall is present between the sinus venosus in the right atrium and the CPV. A deficiency of this wall explains the development of sinus venosus defects.

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.

Comparison of echocardiographic methods with magnetic resonance imaging for assessment of right ventricular function in children.

Assessment of right ventricular (RV) function is clinically relevant in the follow-up of various forms of congenital heart disease. Agreement on the value of different echocardiographic approaches for this purpose is lacking. Magnetic resonance imaging (MRI) provides dimensionally accurate RV volumes and ejection fraction. Transthoracic 2-dimensional echocardiography from 3 different views and gradient-echo tomographic MRI were performed in 16 children with congenital heart disease and 17 age-matched healthy children. RV volumes and ejection fraction were calculated with 5 mono- and biplane area-length and multiple-slice echocardiographic methods. Adequate MRI and echocardiographic apical 4-chamber images could be obtained in all 33 children. The best correlation between MRI and echocardiographic volumes was with the biplane pyramidal approximation method. End-diastolic volume by MRI was 92 +/- 27 ml: systematic difference with echocardiography was +14 +/- 16 ml (r = 0.86). End-systolic volume by MRI was 33 +/- 13 ml: systematic difference with echocardiography was -4 +/- 7 ml (r = 0.82). Ejection fraction by MRI was 65 +/- 8%: systematic difference with echocardiography was +5 +/- 7% (r = 0.72), using monoplane ellipsoid approximation. For all echocardiographic methods, significant effects of RV geometry were noted. Echocardiographic mono- and biplane area-length and multiple-slice calculations demonstrated moderate correlation and significant systematic errors compared with MRI-derived RV volumes. Echocardiographic results were influenced by RV geometry. The relatively simple monoplane area-length method provides ejection fraction results acceptable for clinical practice; results are not improved by more complex biplane and/or multislice methods.

Repair of tricuspid atresia in 100 patients.

One hundred consecutive patients with situs solitus of the atria and tricuspid atresia have undergone surgical repair since 1968. In patients with ventriculoarterial concordance, a nonvalved Dacron conduit or an aortic valve homograft was interposed between the right atrium and right ventricular outlet chamber. In patients with ventriculoarterial discordance, an aortic valve homograft established continuity between the right atrium and pulmonary artery. Among 73 patients with ventriculoarterial concordance, the hospital mortality rate was 11% (eight deaths), and in 27 patients with ventriculoarterial discordance, it was 15% (four deaths). Before 1974, the year of operation was a significant determinant of hospital mortality (p less than 0.001). Thereafter, the hospital mortality declined and is currently 3.7%. In 82 patients with ages ranging from 4 to 16 years, there were six deaths (7.3%) whereas there were six deaths (33.3%) in 18 patients less than 4 or more than 16 years (p less than 0.001). Mode of ventriculoarterial connection and type of repair did not influence significantly the hospital mortality. There were six late deaths due to infection (two), reoperation (two), heart failure (one), and sudden death (one). Regardless of the mode of ventriculoarterial connection, use of a homograft valve produced better results, i.e., more asymptomatic patients (NYHA Class I) (p = 0.0168) and higher postoperative exercise capacity. Postoperative catheterization data and angiocardiographic measurements in patients with ventriculoarterial concordance demonstrated significant advantages with the interposition of a homograft valve between the right atrium and outlet chamber. Of 82 surviving patients, 94% are in NYHA Class I or II.

A modification of staged septation for univentricular atrioventricular connection.

A case is reported in which is described a modification of staged septation for univentricular atrioventricular connection with double-inlet left ventricle and left-sided rudimentary outlet chamber beneath the aorta. This modification diminishes the risk of atrioventricular conduction disturbances by gluing the perforated septation patch on the myocardium in the area overlying the central conduction system. Fixation of the patch was facilitated by exact fitting of the patch in this area, by ample contact of the patch and the myocardium in this area, and by immobilization of the patch because of equal pressures on both sides because of the perforation of the patch. In addition, this modification facilitates evenly drawing in of the ventricular free wall because already, in the first stage, the complete circumference of the patch is fixed, defining the maximal contribution of prosthetic material to the newly created septum.