RESUMEN

OBJECTIVES: To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. METHODS: Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977-1991), intermediate (1992-2006) and late (2007-2021). RESULTS: The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7-30.5). There was a temporal trend towards less shunt palliation (-0.3% per year, 95% CI -0.05 to -0.1). Median age at intracardiac repair was 2.9 years (1.8-5.0), 0.8 years (0.5-1.3) and 0.5 years (0.4-0.7) (P < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (-0.7% per year, 95% CI -0.5 to -1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5-1.0). Survival at 10 years was 79% (64-76), 90% (87-93) and 95% (92-98) (P < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1-6), 12% (8-16) and 21% (13-29) (P < 0.001) in the early, intermediate and late era, respectively. CONCLUSIONS: There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair.

Asunto(s)

RESUMEN

Background and Aims: Postoperative cardiac outcomes after intracardiac repair (ICR) are determined by numerous factors whereas right ventricle (RV) dysfunction is considered essential for them, as only few studies attempted to evaluate it postsurgically. RV's function is supposed to be the strong prognostic factor for patients diagnosed with congenital heart defects; therefore, assessing it is the main objective of the study. Methods: This is a prospective single-centered cohort study performed on 50 pediatric patients with congenital heart disease (CHD) who underwent ICR between January 2019 and January 2022. All patients underwent echocardiographic assessment of RV function via tricuspid annular plane systolic excursion (TAPSE) and fractional area change (FAC) at 1, 24, and 48 h. After surgery, where pre- and postoperative RV pressure, cardiopulmonary bypass (CPB), and aortic cross-clamp (ACC) time were assessed. Similarly ventilation intensive care unit (ICU) and hospital stay times and mediastinal drainage were also monitored. Results: The mean ± standard deviation for pre- and postoperative RV pressure was 49.1 ± 16.12 and 42.7 ± 2.9 mmHg, respectively, whereas that for pre- and postoperative pulmonary artery pressure was 30.4 ± 2.6 and 24.2 ± 12.9 mmHg, with p value of <0.002 and <0.001, respectively. The mean ± standard deviation of CPB and ACC times was 120.92 ± 74.17 and 78.44 ± 50.5 min accordingly, while those for mean ± standard deviation of ventilation time, mediastinum chest drainage, ICU and hospital stays were 30.36 ± 54.04, 43.78 ± 46.7 min, 5.9 ± 4.01 h, were 30.36 ± 54.0, 43.78 ± 46.7 min, 5.9 ± 4.01 and 10.3 ± 4.83 h, respectively. Conclusions: RV dysfunction plays the important role in longer recovery and intraoperative time, while its effect is mostly transient. The use of TAPSE and FAC methods is valuable in the evaluation of postoperative outcomes, and the former proved to be more effective.

RESUMEN

Tetralogy of Fallot is a cyanotic heart disease wherein aortopulmonary collaterals serve as source of pulmonary blood flow to maintain oxygenation. We report an incidentally detected isolated left subclavian artery supplying a compensatory ductus in a child with Tetralogy of Fallot that effectively contributed as a de novo palliative systemic to pulmonary artery shunt. Clinically, the entity could not be suspected, as the child did not have symptoms suggestive of arterial insufficiency of the left arm or weak pulses or neurological symptoms. The child underwent successful intracardiac repair with a reimplantation of left subclavian artery to left common carotid artery.

Asunto(s)

RESUMEN

BACKGROUND: Surgical management has not been encouraged in patients with trisomy 18 (T18) and congenital heart diseases due to poor survival. This study aimed to investigate (1) the appropriateness of palliative surgeries followed by intracardiac repair (ICR) (i.e., two-stage ICR) for patients with a large ventricular septal defect (VSD) and T18, and (2) its impact on their long-term outcomes. METHODS: Medical charts of patients with VSD and T18 who underwent two-stage ICR at the Japanese Red Cross Medical Center between January 2005 and December 2019 were retrospectively reviewed. Demographic data, timing, and types of palliative surgeries, information related to ICR, peri- and postoperative clinical information, postoperative survival, and cause of death were collected. The long-term prognosis of patients treated with two-stage ICR was compared with that of patients treated with primary ICR and palliative surgery without ICR. RESULTS: Overall, 18 (2 male, 16 female) patients underwent two-stage ICR. Pulmonary artery banding was the initial palliative surgery in all patients after a median duration of 19.5 (range 6-194) days of life. The median age and the mean body weight at the time of ICR were 18.2 (7.6-50.7) months and 6.0 ± 1.0 kg, respectively. The mean pulmonary artery pressure and pulmonary vascular resistance index before ICR were 19.1 ± 7.3 mmHg and 3.4 ± 2.0 U m2, respectively. Overall, 17/18 (94%) patients were discharged after ICR. Fourteen (78%) patients were alive during data collection. None of the patients died of cardiac insufficiency, and the median duration of survival was 46.3 (14.3-186.4) months since birth. Most patients required cardiac medications rather than pulmonary vasodilators at the last follow-up. During the study period, three patients underwent primary ICR, and 46 underwent palliative surgery without ICR. Of those who underwent primary ICR, two died in the hospital on the first and 48th day following ICR, and the third died 179 days after the ICR. The Log-rank test revealed a significantly longer survival for the patients treated with two-stage ICR compared with those treated with palliative surgery without ICR (P = 0.003). CONCLUSION: Two-stage ICR improves the long-term survival of patients with VSDs and T18. This safe surgical strategy can also prevent pulmonary hypertension in such patients.

Asunto(s)

RESUMEN

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed.

Asunto(s)

RESUMEN

OBJECTIVE: To evaluate the role of echocardiographic right ventricular function parameters in predicting postoperative vasoactive inotrope requirement after tetralogy of Fallot repair. DESIGN: Prospective observational study. SETTING: A tertiary care hospital. PARTICIPANTS: Fifty-two children undergoing elective intracardiac repair. INTERVENTIONS: Comprehensive transesophageal echocardiography was performed before and after surgery. Fractional shortening, fractional area change, tricuspid annular plane systolic excursion (TAPSE), right ventricular myocardial performance index, tricuspid annular velocities (S', E', A'), and right ventricular global longitudinal strain and strain rate (RV Gls and RV Glsr) were measured. The ratio of peak systolic pressure of the right and left ventricles (Prv/lv) was measured directly from the surgical field pre- and post-repair. The inotrope requirement during first 24 postoperative hours was calculated using the mean Vasoactive-Inotropic Score (VIS). Pearson correlation analysis was used to study the relation between echocardiographic parameters and VIS as well as Prv/lv and VIS. Receiver operating characteristic analysis was used to study the predictive strength of parameters. MEASUREMENTS AND MAIN RESULTS: Among the measured parameters, both pre- and post-repair TAPSE had significant negative correlation with the mean VIS (p < 0.05). Both pre- and post-repair right ventricular myocardial performance index and S', E', A', RV Gls, RV Glsr, Prv/lv also had significant correlation with the mean VIS (p < 0.05). Of these, TAPSE, RV Gls, RV Glsr, and Prv/lv had significant predictive strength (p < 0.05) and reasonable sensitivity and specificity (area under the curve > 0.6) for predicting high mean VIS (VIS > 20). CONCLUSION: Tricuspid annular plane systolic excursion, RV Gls, RV Glsr, and Prv/lv could predict a postoperative high mean VIS with significant strength and reasonable sensitivity and specificity.

Asunto(s)

RESUMEN

We report on two patients who underwent bilateral lung transplantation (BLTx) combined with cardiac surgery. Patient 1 was a female whose pulmonary hypertension resulted from a congenital atrial septal defect (ASD) and idiopathic pulmonary arterial hypertension. She had a very small left ventricle (LV). We initiated venoarterial extracorporeal membrane oxygenation (ECMO) before induction of general anesthesia. She underwent ASD patch closure, pulmonary artery replacement, and BLTx under cardiopulmonary bypass (CPB). At the weaning from CPB, primary graft dysfunction and pulmonary edema induced by LV diastolic dysfunction was apparent. We gradually decreased the ECMO support and eventually weaned off the ECMO on the 4th postoperative day (POD) and the ventilator on the 29th POD. Patient 2 was a male with Eisenmenger syndrome, which resulted from ASD and ventricular septal defect (VSD). He had a normal LV. General anesthesia was induced smoothly without ECMO. He underwent ASD and VSD patch closure, pulmonary artery replacement, and BLTx under CPB. Weaning from CPB proceeded smoothly. These patients needed different management because of their different LV function. Especially, perioperative management of the BLTx patient with LV diastolic dysfunction was difficult. Assessment of perioperative cardiac function is very important in BLTx combined with cardiac surgery.

Asunto(s)

RESUMEN

A 13-year-old girl developed the features of congestive heart failure on day three of intracardiac repair of tetralogy of Fallot. A routine transthoracic echocardiography identified the missed supramitral ring (SMR), closely placed just above the mitral valve, the very reason for severe mitral stenosis and its implication in the present clinical deterioration. A rare association of SMR with tetralogy of Fallot may be missed narrowly because closely placed SMR can sometimes mask its hemodynamic effect when present with significant right ventricular outflow tract obstruction as in this case. .

RESUMEN

Although mild increase in pulmonary vascular resistance following intracardiac repair of tetralogy of Fallot is often seen in the early postoperative period, it usually subsides without any sequel. Persistent severe pulmonary artery hypertension after total correction is rare. We report a child with tetralogy of Fallot and Down's syndrome, who developed severe pulmonary hypertension and low cardiac output syndrome following an intracardiac repair, which was resistant to specific pulmonary vasodilators and increasing ionotropes. The post correction echocardiogram suggested an intact ventricular septal defect patch, no residual gradient across the right ventricular outflow tract, with free pulmonary regurgitation. The child had a poor outcome. A postmortem biopsy revealed histopathological signs of pulmonary hypertension.