Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience.

BACKGROUND: The outcome of prenatally diagnosed tricuspid atresia (TA) is undefined. We sought to characterize clinical and echocardiographic features of fetal TA and to determine factors associated with mortality. METHODS AND RESULTS: All fetuses with TA (n = 88) seen at 3 tertiary care institutions from 1990 to 2005 were reviewed. There were 58 liveborn infants (median gestational age 38 weeks, range 24-40 weeks), 4 in utero demises, 25 terminations of pregnancy, and 1 mother lost to follow-up. Obstruction was present at the pulmonary valve in 27 (45%), aortic valve in 6 (10%), and aortic arch in 15 (25%). Three neonates received compassionate care, 1 died with multiple extracardiac anomalies, 2 were lost to follow-up, and 52 liveborns were actively managed with Blalock-Taussig shunt (23), Norwood palliation (14), pulmonary artery band (10), bidirectional cavopulmonary connection (3), atrial septostomy (1), and right outflow stent (1). Of those actively managed, there were 7 (14%) of 52 who died. Kaplan-Meier estimates of survival were 91% at 1 month, 87% at 6 months, and 83% at 1 year with no subsequent deaths for 13 years. By multivariate analysis, 2 independent factors were associated with an increase in time-related mortality in the actively managed group: presence of chromosomal anomaly or syndrome (P = .005) and use of extracorporeal membrane oxygenation (P = .002). CONCLUSIONS: This is the largest study describing TA in fetus. Compared with published observations of TA diagnosed postnatally, antenatal diagnosis of TA appears to have similar short-term survival in pregnancies surviving to birth.

Absent pulmonary valve, tricuspid atresia, and congenital heart block.

We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed. Cardiac catheterization revealed high cavopulmonary pressures and ventricular dysfunction precluding Fontan completion. Heart transplantation was performed at 3.75 years of age. The patient is alive and well 26 months posttransplantation.

Long-term outcome and quality of life in adult patients after the Fontan operation.

The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative procedure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all patients (n = 36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiography. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 patients. A total of 195 hospital admissions (mean 3.8 +/- 2.7, range 1 to 13) was recorded. Quality-of-life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particular, reoperations, arrhythmias, and thromboembolic events compromised quality of life.

Maternal infant bonding enhanced after atrial septostomy in cyanotic neonates in a general hospital.

UNLABELLED: Seventeen newborns in a general hospital had a successful atrial septostomy when indicated. Mostly done at the bedside under echocardiographical guidance, the successful introduction of this procedure enabled the infants to remain with mother to facilitate feeding and bonding prior to elective transfer to a children's hospital for corrective surgery. BACKGROUND: Atrial septostomy is a well-recognised intervention in the newborn to facilitate atrial mixing in transposition of the great arteries (TGA) or to decompress an atrium where the connecting AV valve is absent or stenosed, e.g. tricuspid atresia (TA). AIMS: To review the outcome of this procedure in a general hospital with appropriate neonatal and cardiological facilities. METHODS: Retrospective review over an 11-year period. RESULTS: Seventeen inborn infants had successful atrial septostomies, 11 with TGA and 6 with TA. All done under general anaesthesia, 15 were performed in the newborn nurseries, under echocardiographic guidance, and 2 in the catheter laboratory. No complications occurred. Eleven had a prenatal diagnosis made. All infants were able to be subsequently nursed by their mothers, affording prime time facilitating feeding and bonding. They were electively transferred to a children's hospital for corrective surgery. CONCLUSIONS: Atrial septostomy can be safely performed in a general hospital with appropriate neonatal and cardiological expertise. Such intervention allows for elective transfer of the infant for corrective surgery, allowing the infant and mother to be initially cared for at the one hospital, thereby facilitating maternal contact, feeding and bonding, doing away with the added stress of emergency transfer.

Stent implantation in a central aorto-pulmonary shunt.

A 5.5-week-old infant with tricuspid atresia presented with severe hypoxemia not responding to the placement of a central shunt (4 mm polytetrafluorethylene). The infant was taken to the catheterization laboratory, where an AVE stent was successfully implanted in a severe postoperative stenosis, at the pulmonary end of the anastomosis. The oxygen saturation improved significantly, however, the infant died due to renal failure. The post-mortem anatomical findings are shown.

Cardiac emergencies.

The diagnosis and management of pediatric cardiac emergencies can be challenging and complicated. Early presentations are usually the result of ductal-dependent lesions and appear with cyanosis and shock. Later presentations are the result of volume overload or pump failure and present with signs of congestive heart failure. Acquired diseases also present as congestive heart failure or arrhythmias.

Terapia de ressincronização cardíaca em portador de coração univentricular para correção de dissincroniomiopatia causada por mudança do sítio de estimulação / Cardiac resynchronization therapy in severe systolic dysfunction in a univentricular heart patient to correct dyssynchrony cardiomyopathy caused by change of stimulation site

Paciente com 6 anos de idade, do sexo masculino, portador de coração univentricular decorrente deatresia tricúspide com hipoplasia de ventrículo direito tipo IIC e submetido a implante de marcapasso por bloqueio atrioventricular total no pós-operatório de cirurgia de Fontan, em 2012. Houve necessidade de troca do sistema de estimulação em decorrência de infecção de loja logo após a troca do marcapasso, em 2014. Seis meses após amudança do sítio de estimulação, começou a apresentar quadro de insuficiência cardíaca refratária e disfunção sistólica grave, e o transplante cardíaco foi indicado. O paciente foi submetido a terapia de ressincronização cardíaca orientada por eletrocardiografia triaxial como ponte para transplante e apresentou melhora clínico-estrutural após10 semanas, recebendo alta hospitalar...

We report the case of a 6-year-old male patient with univentricular heart due to tricuspid atresia with right ventricular hypoplasia (IIc), who had a pacemaker implanted for complete heart block after a Fontan surgery in 2012. The stimulation system had to be exchanged due to a pocket infection soon after the pacemaker was exchanged in 2014. Six months after the stimulation site was exchanged he presented refractory heart failure and severe systolic dysfunction and a heart transplantation was indicated. He was submitted to cardiac resynchronization therapy guided by triaxial electrocardiography as a bridge for transplantation. The patient presented clinical and structural improvement after 10 weeks and was discharged...

Anesthetic care during modified fontan procedure without cardiopulmonary bypass.

OBJECTIVE: The purpose of this study was to report the anesthetic care of patients during performance of a Fontan procedure without cardiopulmonary bypass (CPB). DESIGN: Retrospective chart review. SETTING: Operating room of a university hospital. PARTICIPANTS: Seven pediatric-patients undergoing inferior vena cava (IVC)-to-pulmonary artery (PA) anastomosis for completion of the Fontan procedure. INTERVENTIONS: Charts were reviewed for anesthetic technique, hemodynamic and ventilatory changes occurring during the procedure, and anesthetic interventions that were provided. MEASUREMENTS AND MAIN RESULTS: The off-bypass Fontan procedure was attempted in 7 patients (age: 26 months-7 years, weight: 13 to 28 kg). Exposure of the PA was not feasible in 1 patient because of a markedly enlarged right atrium. In the remaining 6 patients, before cross-clamping of the PA to allow for the proximal anastomosis between the PA and the conduit, alkalosis (pH > or =7.5) was maintained by the administration of sodium bicarbonate. After PA cross-clamping, fluid administration was necessary in 5 patients and dopamine (3-7 microg/kg/min) was necessary in 4 patients. The minute ventilation was increased by 18 +/- 7% to maintain baseline PaCO2 values. Before the placement of the PA cross-clamp, the end-tidal PaCO2 difference was 7 +/- 4 mmHg and the transcutaneous (TC)-PaCO2 difference was 3 +/- 2 mmHg. The end-tidal PaCO2 difference increased to 14 +/- 6 mmHg during cross-clamping of the PA, whereas no change was noted in the TC-PaCO2 difference. Once the proximal anastomosis was completed, a bridge was placed to redirect blood from the IVC to the right atrium while the IVC was clamped and attached to the distal end of the conduit. After placement of the distal end of the bridge into the IVC, fluid administration to maintain the blood pressure was necessary in 3 patients. In 1 patient, 20 minutes after placement of the bridge, the authors noted a progressive increase in the central venous pressure reading measured from the left femoral vein and the need for the administration of volume to maintain the mean arterial pressure. Examination of the bridge revealed occlusion with thrombus despite an activated coagulation time value of 250 to 300 seconds. The tracheas of 3 of the 6 patients were extubated in the operating room, whereas the other 3 were extubated in the pediatric intensive care unit within 4 hours of completion of the procedure. The 1 patient who required the use of CPB required reintubation and had a protracted intensive care unit course. The other 6 patients were discharged home on postoperative days 7 to 12. CONCLUSIONS: With alteration of the anesthetic technique, the Fontan procedure can be performed in selected patients without the need for CPB.