Prenatal identification of the pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia.

OBJECTIVE: To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia. BACKGROUND: In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management. METHODS: We carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings. RESULTS: Of 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced. CONCLUSION: The patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.

Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors.

BACKGROUND: This study was conducted to evaluate surgical outcome and to identify risk factors for hospital mortality and reoperation after repair of complete atrioventricular septal defect. METHODS: A total of 147 consecutive children underwent repair between January 1986 and December 1998. Of those, 106 had Down syndrome, 37% had normal chromosomes, and 4 had other syndromes; 108 underwent primary repair, 19 had prior pulmonary artery banding, and 20 had additional tetralogy of Fallot. The median weight at primary repair was 4.5 kg. A two-patch technique was used in 88%. RESULTS: The 30-day mortality was 15% (70% confidence interval [CI] 12% to 19%). A double orifice atrioventricular valve was found to be a significant risk factor (p = 0.002), with 6 of 11 patients dying. If double orifice atrioventricular valve patients are excluded, the mortality rate falls to 12% (70% CI 9% to 15%). No difference in mortality was found between Down syndrome and chromosomally normal children but the latter more commonly required reoperation. Chromosomally normal children frequently have a dysplastic common atrioventricular valve (24% versus 3% in Down children, p < 0.001). In a multivariate Cox model including both variables, the presence of a dysplastic atrioventricular valve was a significant risk factor for reoperation. After controlling for the presence of a dysplastic atrioventricular valve, Down syndrome retained a significant protective effect but the upper limit of the confidence interval was close to 1. CONCLUSIONS: The presence of a double orifice atrioventricular valve emerged as an unforeseen risk factor for death.

The left atrioventricular valve in partial atrioventricular septal defect: management strategy and surgical outcome.

OBJECTIVE: To test the hypothesis that in patients with a partial atrioventricular septal defect (PAVSD) and a competent left atrioventricular valve (LAVV), sutures should be placed across the line of apposition of the superior and inferior bridging leaflets, septal commissure (SC), to prevent the development of regurgitation. Outcome of surgery and risk factors for the need for LAVV reoperation of patients with mild or no LAVV regurgitation (LAVVR) were evaluated. BACKGROUND: Controversy over management of the LAVV in PAVSD. METHOD: One hundred and forty seven children with PAVSD underwent surgical repair at the Royal Brompton Hospital between January 1983 and December 1999. Of this group, 21 (16.7%) had LAVVR of sufficient severity to require surgical intervention and were therefore excluded from analysis. The median age and weight at repair of those with mild or no LAVVR was 4.1 years and 15.4 kg. One hundred and eight had normal chromosomes, 13 Down syndrome and five other syndromes. The interatrial communication was closed using a pericardial patch in 62.7% and with synthetic material in the remainder. Intraoperative testing of LAVV competence was undertaken using saline injection into the left ventricle. In 80.9%, sutures were placed across the line of apposition of the left sided superior and inferior bridging leaflets partially to close the SC (sometimes incorrectly named the mitral valve cleft). RESULT: The overall hospital mortality was 3.2% (95% confidence interval (CI) 1, 8.4%), which did not differ statistically in the last 20 years. No specific risk factors for early death were identified. Eleven patients (8.7%, 95% CI 4.7, 15.4%) required reoperation, 10 for LAVV repair and 1 resection of subaortic stenosis. Univariate analysis of risk factors for LAVV reoperation were low weight, relatively small size LAVV, the presence of a small preoperative interventricular interchordal communication and duration of ventilation. Ten (9.8%) of 102 patients in whom SC was sutured required LAVV reoperation but none for 24 in whom the commissure was left alone. CONCLUSION: The hypothesis that in the absence of preoperative LAVVR it is necessary to place sutures in the SC has not been proven. We consider that in addition to preoperative cross sectional echocardiographic assessment of LAVVR intraoperative evaluation of LAVV function allows discrimination between those valves where sutures to the septal commissure are necessary and those where the valve can be left undisturbed.

Evaluation of fetal arrhythmias from simultaneous pulsed wave Doppler in pulmonary artery and vein.

OBJECTIVE: To evaluate the clinical application of simultaneous recordings of pulsed wave Doppler (PWD) signals in pulmonary artery and vein as alternative sampling site for assessment of arrhythmias in the fetus. DESIGN: Prospective, cross-sectional study. SETTING: Tertiary referral centre for fetal cardiology. PATIENTS AND METHODS: From July 1999 to July 2005 PWD was used in pulmonary vessels to assess fetal arrhythmias at 15-40 weeks' gestation. Sample volume placement in the peripheral lung vessels was guided by colour flow mapping on a four-chamber section of the fetal heart. Atrial and ventricular systoles were identified from the pulmonary venous and arterial signals respectively. M-mode recordings were used for comparison. OUTCOME MEASURES: Diagnosis of fetal arrhythmias. RESULTS: Of 129 cases, 15 had supraventricular tachycardia, 12 with 1:1 atrioventricular conduction and 3 with atrial flutter and 2:1 block. There were 96 cases of atrial and 7 of ventricular premature beats, 2 of sinus bradycardia, 8 of variable degree heart block and 1 of ventricular tachycardia. PWD was diagnostic in 119 cases. PWD was better than M mode for diagnosis of premature beats and added information about mechanisms of tachycardia. Both methods facilitated interpretation of all arrhythmia patterns, although PWD was of less practical value in cases of complete heart block. CONCLUSION: Simultaneous PWD recording of pulmonary vessels in the fetus allows accurate diagnosis of arrhythmias. It is easily obtained with standard ultrasound equipment and adds to the armamentarium of diagnostic techniques for assessment of rhythm abnormalities prenatally.

Assessment of operability for common arterial trunk without cardiac catheterisation.

Pulmonary vascular disease is a risk factor for the surgical management of common arterial trunk. Surgical repair, therefore, is usually performed in early infancy, before irreversible changes can occur in the epulmonary vasculature. Because of this, there has been an increasing tendency to dispense with cardiac catheterisation as a means of assessing pulmonary vascular disease. Cardiac catheterisation, nonetheless, is still performed when there is a risk of pulmonary vascular disease, such as in older children. There are no clear guidelines, however, as to who should be catheterised. We have developed a simple screening test to help make this decision.

Outcome of the construction of a Blalock-Taussig shunt in adolescents and adults.

AIMS: The purpose of our study was to ascertain the outcome of the construction of a Blalock-Taussig shunt in patients aged 12 years and over. PATIENTS AND METHODS: We identified 21 patients in whom a Blalock-Taussig shunt had been constructed subsequent to the age of 12 years. Of the patients, 9 were female, and their median age was 18.5 years, with a range from 12 to 46 years. All had usual atrial arrangement, and the atrioventricular connections were concordant in 11, with univentricular atrioventricular connection in 10. Pulmonary atresia was present in 8 (38 per cent), and pulmonary stenosis, either valvar or subvalvar, in 13. An interposition graft had been placed between the subclavian and pulmonary arteries in 16 patients, and an end-to-side anastomosis between the arteries in 5. RESULTS: One patient had died in hospital, while 4 patients had died during the period of follow-up after initial construction of the shunt. Long-term follow-up was available in 86 per cent of patients. In the 3 patients lost to follow-up, the shunt had been known to be functioning at periods of 4, 8, and 10 years, respectively. Actuarial freedom from death after a period of 17 years was 76 per cent. In 8 patients, a period of 10 years had elapsed with the shunt patent, and a further 10 had a patent shunt after 5 years follow-up. Symptomatic improvement was reported in 16 (76 per cent) patients, although adverse cardiac events had occurred during follow-up in 17, including congestive heart failure in 3, atrial fibrillation in 3, and endocarditis in 2. In 2 patients, it had been possible to proceed to biventricular repair, one with tetralogy of Fallot, and the other having a Rastelli procedure. Further in 3 patients, it had been possible to construct the Fontan circulation, or one of its variants. One patient has undergone cardiac transplantation, while 2 are awaiting transplantation. CONCLUSIONS: Symptomatic improvement can be achieved by construction of a Blalock-Taussig shunt in older subjects, and the risks of surgery are low. Later repair may be feasible in some patients, but adverse cardiac events may follow the increased volume load on the systemic ventricle.

A tunnel from the left sinus of Valsalva to the right atrium.

We describe an individualised approach in the rare situation of a tunnel between the left aortic sinus of Valsalva and the junction of the superior caval vein with the right atrium. It is necessary, in this rare anomaly, to identify the origin of the left coronary artery prior to intervention.

Covert video surveillance continues to provoke debate.

When the editor invites comment on a response to an analysis of a criticism of a protocol already defended by the author in this journal, the issue is clearly contentious. I will comment briefly on Thomas's paper in this issue of the journal, and look at points of agreement as well as dissent.

Origem anômala da artéria coronária esquerda do tronco pulmonar. Perfil clínico r resultados a médio prazo do tratamento cirúrgico / Anomalous origin of the left coronary artery from the pulmonary trunk. Clinical features and midterm results after surgical tratment

Objetivo - Relatar a experiência com a origem anômala da artéria coronária esquerda (OACE) do tronco pulmonar, enfatizando-se dados pré-operatórios, cirúrgicos e os do seguimento a médio prazo. Métodos - Análise retrospectiva em 11 pacientes operados no Royal Brompton Hospital no período de outubro/84 a abril/97. Resultados - Nove lactentes estavam em insuficiência cardíaca (IC) e duas outras crianças apresentavam-se com dispnéia e dor precordial, todos com alterações eletrocardiográficas. O ecocardiograma identificou a origem coronária anômala em 7 (64 por cento) pacientes e o estudo hemodinâmico foi realizado em 7 casos. Todos os lactentes foram operados entre o 2§ e o 10§ mês de vida. Seis pacientes foram submetidos a reimplante da artéria coronária esquerda na aorta, enquanto cinco casos foram operados através da técnica de Takeuchi. Todos os pacientes estão vivos, com evidente melhora eletrocardiográfica e da função ventricular. Reoperação foi necessária em dois pacientes operados pela técnica de Takeuchi devido à estenose supravalvar pulmonar grave. Conclusão - A maioria desses pacientes com ACE apresentam-se precocemente com IC grave associada a alterações eletrocardiográficas. O tratamento cirúrgico deve ser realizado precocemente, de preferência através de implante da coronária anômala na aorta, com grande possibilidade de sucesso. A melhora deve ser esperada a curto prazo após a cirurgia, assim como a normalização da função ventricular esquerda.