Can atrioventricular septal defects exist with intact septal structures?

The hallmark of an atrioventricular septal defect (AVSD) is a common atrioventricular junction, giving rise to a trileaflet left atrioventricular valve. AVSDs have the potential for interatrial shunting alone, interventricular shunting alone, or both. AVSDs without interatrial or interventricular communications have been identified at postmortem examination, but there are no reports of AVSDs with intact septal structures diagnosed in life. Six patients are described with AVSD and intact atrial and ventricular septa diagnosed echocardiographically. This report shows that AVSDs can exist without interatrial or interventricular communications and that the characteristic feature of this condition, the common atrioventricular junction with a trileaflet left atrioventricular valve, can be diagnosed in life by using cross sectional echocardiography. AVSDs with intact septal structures may be more common than previously described.

Complications of supra-annular mitral valve placement in infants.

Two infants underwent supra-annular placement of prosthetic mitral valves. The objective of this strategy was to insert a larger valve and delay replacement. This approach was initially successful but by two and three years later the patients developed impairment of cardiac function. The prosthesis decreased the volume and compliance of the left atrium causing high left atrial and pulmonary venous pressures. The "ventricularised" atrium below the prosthesis dilated. In neither case was it possible to delay second valve replacement.

Clinical impact of first and early second trimester fetal echocardiography on high risk pregnancies.

OBJECTIVE: To evaluate the clinical impact of fetal echocardiography before 16 weeks' gestation on the management of pregnancies with fetuses at risk of congenital heart disease. DESIGN AND SETTING: Observational study in a fetal medicine unit. PARTICIPANTS AND METHODS: 222 consecutive women with high risk pregnancies (230 fetuses) underwent transabdominal fetal echocardiography at a median gestation of 14(+1) weeks. For 10%, transvaginal scans were also performed. Criteria for normal scans were normal sequential segmental analysis, symmetrical four chamber view, normal semilunar valves, arterial outflow tracts, and ductal and aortic arches. Early scans were compared with mid-second trimester fetal echocardiography. Postmortem and postnatal data were added. Adverse pregnancy outcomes were diagnosis of a major cardiac or extracardiac abnormality, chromosomal defects, intrauterine death, or termination of pregnancy. RESULTS: There were 21 abnormal cardiac scans (9%): 14 major structural defects (eight isolated, six with chromosomal or extracardiac abnormalities) in pregnancies resulting in three live births, one intrauterine death, and 10 terminated pregnancies. Seven scans showed asymmetry between right and left sided structures (two isolated, five with chromosomal or extracardiac abnormalities); six of the seven pregnancies were terminated. The scans were normal in 199 cases (87%). Cardiac follow up of 184 of 199 babies (93%) confirmed situs and connections. One case each of pulmonary stenosis and ventricular septal defect requiring postnatal intervention were diagnosed at later scans. In 28 of 199 (14%) babies there was a non-cardiac adverse outcome. First examination was not diagnostic for 10 (4%). CONCLUSION: Early fetal echocardiography in high risk pregnancies was diagnostic in 96%. Abnormal cardiac scans (isolated in 48%, major structural defects in 67%) led to termination of pregnancy in 76%. Most cardiac scans were normal, allowing family reassurance. The high number of adverse outcomes with normal cardiac anatomy stresses the need for a multidisciplinary approach to early fetal echocardiography.

Improving the effectiveness of routine prenatal screening for major congenital heart defects.

OBJECTIVE: To evaluate the effectiveness of adding outlet views to the four chamber view in routine prenatal ultrasound screening for major congenital heart defects (CHD) as performed by trained sonographers, and to compare the procedure with current practice. DESIGN AND SETTING: Prospective observational study at a London teaching hospital. PARTICIPANTS AND METHODS: 9277 women booked at a single institution (80% had first trimester nuchal translucency measurement) due to have routine fetal cardiac screening using the four chamber and outflow tract views at > 18 weeks of gestation. MAIN OUTCOME MEASURE: Identification of major CHD in chromosomally normal and abnormal pregnancies antenatally or postnatally. RESULTS: There were 40 abnormalities (4.3/1000), of which 30 were chromosomally normal (3.3/1000). The overall antenatal detection rate was 75% (95% confidence interval (CI) 59% to 87%) and 70% (95% CI 51% to 85%) for euploid pregnancies. Abnormal cardiac views accounted for 70% of all prenatal diagnoses, 30% of which were made at < or = 18 weeks. The sensitivity of cardiac views during the first scan at > 18 weeks was 52%. Of all patients undergoing nuchal translucency screening, 34 had major CHD, nine with increased nuchal translucency (26.5%). Factors influencing the results of this screening programme were training and audit of operators, adequate equipment for antenatal examination, ease of access, and low threshold for referral to specialised fetal echocardiography. CONCLUSION: Adding ventricular outlet views to the four chamber assessment of the heart at routine fetal anomaly scans at > 18 weeks is the most effective technique to detect CHD prenatally. The success of such a programme depends on an infrastructure committed to continuous in house training of obstetric ultrasonographers coupled with feedback from specialised fetal cardiologists, as well as adequate resource allocation to obstetric hospitals involved with antenatal screening.

Fetal, infant, and childhood growth and adult blood pressure: a longitudinal study from birth to 22 years of age.

BACKGROUND: People who are small at birth tend to have higher blood pressure in later life. However, it is not clear whether it is fetal growth restriction or the accelerated postnatal growth that often follows it that leads to higher blood pressure. METHODS AND RESULTS: We studied blood pressure in 346 British men and women aged 22 years whose size had been measured at birth and for the first 10 years of life. Their childhood growth was characterized using a conditional method that, free from the effect of regression to the mean, estimated catch-up growth. People who had been small at birth but who gained weight rapidly during early childhood (1 to 5 years) had the highest adult blood pressures. Systolic pressure increased by 1.3 mm Hg (95% CI, 0.3 to 2.3) for every standard deviation score decrease in birth weight and, independently, increased by 1.6 mm Hg (95% CI, 0.6 to 2.7) for every standard deviation score increase in early childhood weight gain. Adjustment for adult body mass index attenuated the effect of early childhood weight gain but not of birth weight. Relationships were smaller for diastolic pressure. Weight gain in the first year of life did not influence adult blood pressure. CONCLUSIONS: Part of the risk of adult hypertension is set in fetal life. Accelerated weight gain in early childhood adds to this risk, which is partly mediated through the prediction of adult fatness. The primary prevention of hypertension may depend on strategies that promote fetal growth and reduce childhood obesity.

Abnormalities of right ventricular long axis function after atrial repair of transposition of the great arteries.

BACKGROUND: While volume derived global indices of right ventricle (RV) function are frequently abnormal after the Mustard procedure, the mechanism for these abnormalities is poorly understood. RV muscle fibres are predominantly arranged longitudinally and thus indices derived in the long axis may better describe RV function. METHODS: 20 survivors of the Mustard operation were studied (age 7.8-37.3 years, median 14.2 years). Long axis recordings from the apical four chamber view were obtained with the M mode cursor positioned through the lateral angle of the tricuspid valve annulus. M mode traces were recorded on paper and later digitised to derive total atrioventricular ring excursion, peak lengthening rate, and peak shortening rate. These data were averaged and compared with control data for the normal RV and left ventricle (LV). RESULTS: RV total atrioventricular ring excursion was lower than that for the RV (p < 0.0001) or LV (p < 0.005) of controls. Peak lengthening rate was lower than the normal RV (p < 0.0001) and LV (p < 0.0001) rates. Furthermore, peak shortening rate was less than that of normal RV (p < 0.0001) and normal LV (p < 0.005) controls. CONCLUSION: Systemic RV long axis function is notably reduced compared with that of either the normal subpulmonary RV or the systemic LV. This presumably reflects the response of the predominantly longitudinally arranged myocardial fibres to increased afterload. However, such measurements may provide a more sensitive marker for progressive changes in global function during long term follow up.

Long-term results of relief of subaortic stenosis in univentricular atrioventricular connection with discordant ventriculoarterial connections.

BACKGROUND: We set out to examine the long-term results of relief of subaortic stenosis by enlargement of ventricular septal defect in patients with univentricular atrioventricular connection to a dominant left ventricle and discordant ventriculoarterial connections. METHODS: Twenty-four patients underwent enlargement of ventricular septal defect between 1985 and 1998 at a median age of 3.2 years (range, 3 weeks to 14 years). Ten patients were younger than 1 year of age. Eighteen had undergone previous banding of the pulmonary trunk, 9 of whom also required repair of coarctation of the aorta. The median subaortic gradient before enlargement was 46 mm Hg. Twenty-three patients had a patch to enlarge the rudimentary right ventricle. RESULTS: Five patients (21%) died in the early postoperative period. The overall survival at 1 and 3 years was 73%, and at 5 and 10 years was 68% and 60%, respectively. Complete heart block requiring insertion of a pacemaker occurred in 2 patients (8%). A Fontan operation was performed in 10 patients, 5 underwent a bidirectional Glenn procedure, and 2 required cardiac transplantation. Follow-up was complete in all survivors at a median time of 6.7 years (range, 8 months to 13 years). From the earlier part of the series, 3 patients experienced aortic insufficiency and 2 had recurrent obstruction. Factors adversely affecting survival were age younger than 1 year at operation and presence of obstruction within the aortic arch. CONCLUSIONS: Our experience shows that, in patients with univentricular atrioventricular connection to a dominant left ventricle and subaortic stenosis, enlargement of the ventricular septal defect provides satisfactory relief of obstruction except in those younger than 1 year of age, and those who have associated obstruction in the aortic arch.

Rupture of pulmonary aneurysms in association with long-standing Waterston shunts.

Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinicopathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.

Are infant behavioural feeding difficulties associated with congenital heart disease?

AIM: To compare the feeding patterns and difficulties of infants with congenital heart disease (CHD) and healthy controls. Information was gathered via parental questionnaires. METHODS: A matched case controlled study of 64 infants with CHD compared with 64 healthy controls. RESULTS: The main findings were: (1) Feeding patterns: mothers with infants with CHD used bottle-feeding as a first method of feeding their babies more often (CHD, 20%, controls, 2%); (2) Specific feeding difficulties: (a) infants with CHD were significantly more breathless when feeding (CHD = 16%, controls, 0%), (b) had more vomiting at mealtimes (CHD = 23%, controls = 11%), but (c) had significantly less spitting (CHD = 19%, controls, 41%); and (3) infants with CHD showed significantly reduced growth. CONCLUSIONS: The feeding difficulties are related to the organic condition and not specific difficulties in mother-infant interaction. Professional support may be required for mothers of infants with CHD to maintain feeding routines and to deal with the difficulties that arise.

Right and left ventricular long-axis function in the fetus using angular M-mode.

OBJECTIVE: Long-axis function is determined by the longitudinally oriented myocardial fibers. Postnatally, conventional M-mode is used to assess tricuspid and mitral valve ring movements in relation to the cardiac apex. During fetal life, this is precluded by variable fetal position. We assessed the feasibility of determining right and left ventricular long-axis function in the fetus. METHODS: A prospective, pilot study. The four-chamber view obtained during routine fetal echocardiography was recorded in a cineloop to which B-mode guided M-mode echocardiography was applied using angular M-mode. This allowed retrospective and correct placement of the cursor line from cardiac apex to tricuspid or mitral valve rings. M-mode tracings of the valve ring movements in relation to the apex were derived from the originally stored loop. Data from 18 fetuses (17-29 weeks of gestation) were available for analysis. Total excursion of the valve rings was measured offline. A second cursor line was simultaneously placed in the left ventricular outflow tract during color flow mapping for timing purposes (n = 6). RESULTS: Right and left ventricular long-axis recordings were obtained in 18 and 14 cases, respectively. Total right ventricular excursion was 5.2 mm (SD, 0.9 mm) (range, 3.9-7.2 mm). Total left ventricular free wall excursion was 4.5 mm (SD, 1.1 mm) (range, 3.0-6.8 mm). For paired data, the mean of differences (right ventricle-left ventricle) was 0.8 mm (95% confidence interval 0.5-1.2). The valve rings moved towards the apex during systole (shortening) and away from it during diastole (lengthening). Peak downward movement coincided with cessation of aortic flow and diastolic lengthening with flow through the atrioventricular valves. CONCLUSION: Long-axis function in the fetus is feasible if M-mode angle correction is used. There was no clinically significant difference between tricuspid and mitral valve excursions. The pattern of atrioventricular valve movement is coordinate and similar to that of adults. Long-axis function offers a new avenue to study systolic and diastolic function in the fetus.

Cardiac complications in children following infection with varicella zoster virus.

Infection with varicella zoster virus, leading to chicken pox in susceptible hosts, is usually a benign self-limiting disease conferring immunity in those affected. Cardiac complications are rare, but when present may lead to severe morbidity or mortality. We have recently encountered three children, all of whom developed significant cardiac complications secondary to infection with varicella. Myocarditis has long been associated with such infection. The pathological mechanism is presumed similar to other cardiotropic viruses, where both direct cytopathic and secondary auto-immune effects contribute to myocardial cellular destruction and ventricular dysfunction. Complications include arrhythmias and progression to dilated cardiomyopathy. Pericarditis, and secondary pericardial effusion, related to infection with the virus is most commonly associated with secondary bacterial infiltration. Both cardiac tamponade and chronic pericardial constriction may result. Endocarditis complicating varicella has only been described in the last fifteen years, and is associated with the emergence of virulent strains of both streptococcus and staphylococcus, the two organisms most commonly associated with endocarditis. The exact mechanism by which varicella causes secondary bacterial endocarditis remains unclear. Whilst cardiac complications of infection with the varicella zoster virus are rare, the resulting complications are potentially life threatening. Evidence of varicella-induced carditis must be aggressively pursued in any child with signs of acute cardiac decompensation in whom chicken pox is confirmed or suspected.

Subclavian flap angioplasty: does the arch look after itself?

OBJECTIVES: We sought to assess the early and long-term results of subclavian flap angioplasty in neonates and infants, with particular attention to growth of the hypoplastic arch. METHODS: A retrospective analysis of 185 consecutive patients who underwent subclavian flap angioplasty between 1974 and 1998 was carried out. The patients included 125 neonates and 60 infants, with a median age of 18 days. Sixty-six (36%) patients had an additional ventricular septal defect, 41 (22%) patients had aortic arch hypoplasia diagnosed preoperatively, 141 (76%) had an associated patent ductus arteriosus, and 41 (22%) had additional complex heart disease. Follow-up was with transthoracic Doppler echocardiography in all patients. RESULTS: The early mortality was 3%. Recoarctation, defined as a Doppler gradient of 25 mm Hg or more, occurred in 11 (6%) patients at a median follow-up of 6.2 years (6.2 +/- 4.6 years). This included 4 of the 41 patients in whom arch hypoplasia was diagnosed preoperatively. There were no complications with the left arm. By multivariate analysis, risk factors for death were determined to be residual arch hypoplasia and low birth weight. The only risk factor for recoarctation was persistent arch hypoplasia after surgical treatment. However, angiographic imaging of the aorta showed that recoarctation was not due to a hypoplastic transverse arch, and it was probably at the site of ductal tissue. Survival at 5 and 10 years was 98% and 96%, respectively. Freedom from reoperation for recoarctation at 2 years was 95%, and at 5, 10, and 15 years, it was 92%. CONCLUSIONS: Subclavian flap repair remains an effective technique for repair of aortic coarctation with excellent results and low mortality. In the majority of patients, arch hypoplasia regresses after this procedure.

Anomalous origin of the left coronary artery from the pulmonary trunk. Clinical features and midterm results after surgical treatment.

OBJECTIVE: To report the authors' experience with the anomalous origin of the left coronary artery (AOLCA) from the pulmonary trunk, emphasizing preoperative data, surgical aspects and midterm results of the follow-up. METHODS: Retrospective analysis of 11 patients operated upon at the Royal Brompton Hospital from October, 84 to April, 97. RESULTS: Nine infants had heart failure (HF) and two other children presented with dyspnea and chest pain. All had ECG changes. The echocardio-gram identified the anomalous origin of the coronary artery in 7 (64%) patients and hemodynamic studies were performed in 7 patients. All infants were operated upon between the 2nd and 10th month of life. Six patients were treated with aortic reimplantation of the left coronary artery, whereas five were operated upon according to the Takeuchi technique. All patients are alive, with clear improvement of the ECG changes and ventricular function, as evaluated by echocardiography. Two patients operated upon according to the Takeuchi technique required additional surgery due to severe supravalvular pulmonary stenosis. CONCLUSION: AOLCA is a rare disease. Most patients show early signs of severe HF associated with ECG findings. Surgical therapy must be instituted early in the disease, preferentially through aortic implantation of the anomalous coronary artery, with a high possibility of success. Shortly after surgery, clinical and ECG improvement, as well as normalization of left ventricular function, should be expected.

L-arginine and substance P reverse the pulmonary endothelial dysfunction caused by congenital heart surgery.

BACKGROUND: The increase in pulmonary vascular resistance (PVR) seen in children after cardiopulmonary bypass has been attributed to transient pulmonary endothelial dysfunction (PED). We therefore examined PED in children with congenital heart disease by assessing the L-arginine-nitric oxide (NO) pathway in terms of substrate supplementation (L-arginine [L-Arg]), stimulation of endogenous NO release (substance P [Sub-P]), and end-product provision (inhaled NO) before and after open heart surgery. METHODS AND RESULTS: Ten patients (aged 0.62+/-0.27 years) with pulmonary hypertension undergoing cardiac catheterization who had not had surgery and 10 patients (aged 0.65+/-0.73 years) who had recently undergone cardiopulmonary bypass were examined. All were sedated and paralyzed and received positive-pressure ventilation. Blood samples and pressure measurements were taken from catheters in the pulmonary artery and the pulmonary vein or left atrium. Respiratory mass spectrometry was used to measure oxygen uptake, and cardiac output was determined by the direct Fick method. PVR was calculated during steady state at ventilation with room air, during FIO(2) of 0.65, then during additional intravenous infusion of L-Arg (15 mg. kg(-1). min(-1)) and Sub-P (1 pmol. kg(-1). min(-1)), and finally during inhalation of NO (20 ppm). In preoperative patients, the lack of an additional significant change of PVR with L-Arg, Sub-P, and inhaled NO suggests little preexisting PED. Postoperative PVR was higher, with an additional pulmonary endothelial contribution that was restorable with L-Arg and Sub-P. CONCLUSIONS: Postoperatively, the rise in PVR suggested PED, which was restorable by L-Arg and Sub-P, with no additional effect of inhaled NO. These results may indicate important new treatment strategies for these patients.

Does the modified Blalock-Taussig shunt cause growth of the contralateral pulmonary artery?

BACKGROUND: Although some pediatric cardiology departments have a policy of adopting primary correction of tetralogy of Fallot in all symptomatic infants, we and others still palliate neonates and infants. Effective palliation should ameliorate symptoms and allow growth of the pulmonary arteries. Although studies on the growth of the ipsilateral and contralateral pulmonary arteries after a classic Blalock-Taussig shunt have been reported, pulmonary artery growth after a modified Blalock-Taussig shunt has not been studied as thoroughly. Therefore, we examined whether there is equal growth of the contralateral pulmonary artery after a modified Blalock-Taussig shunt. METHODS: We retrospectively analyzed the records of 140 patients with symptomatic tetralogy of Fallot who had a modified Blalock-Taussig shunt between October 1985 and October 1995. The median age at the time of the Blalock-Taussig shunt was 1.6 months. All patients had corrective procedures at a median age of 1.7 years. Cineangiography was done before the corrective procedure. From the angiograms the diameter of the right and left pulmonary arteries before their first lobar branches and the diameter of the descending thoracic aorta at the level of the diaphragm were measured. For each patient the ratios of right pulmonary artery to descending thoracic aorta and left pulmonary artery to descending thoracic aorta were determined and compared using Student's t test. RESULTS: Of the 140 patients, 114 had a left-sided Blalock-Taussig shunt, 20 had a right-sided shunt, and 6 patients had bilateral shunts. The mean right pulmonary artery to descending thoracic aorta ratio was 1.10 and the mean left pulmonary artery to descending thoracic aorta ratio was 0.98. This difference was not significant. CONCLUSION: We showed equal growth of the right and left pulmonary arteries with no distortion after a modified Blalock-Taussig shunt. If palliation is considered, the modified Blalock-Taussig shunt remains our choice.

Familial atrial standstill with coexistent atrial flutter.

A child with familial atrial standstill and a ventricular pacemaker had syncope due to atrial flutter that was treated by His-bundle ablation. Bradycardia protection alone may be insufficient in patients with atrial standstill.

Exercise echocardiography in children with Kawasaki disease: ventricular long axis is selectively abnormal.

In asymptomatic children with Kawasaki disease, left ventricular traditional markers of systolic and diastolic function are maintained. However, long-axis function, which represents the subendocardium, is abnormal during stress, particularly in patients with versus without coronary aneurysm.