Critical Cyanosis in a Neonate With Unusual Congenital Tricuspid Valve Lesion.

We describe a case of a low birth weight neonate who presented on second day of life with progressive cyanosis and oxygen saturation of 60% by pulse oximetry. The echocardiography examination revealed a large tumor-like mass connected to the tricuspid valve, with severe obstruction of the right ventricular inflow and massive right-to-left shunt through the distended foramen ovale. A large vegetation-like lesion with calcifications was discovered intraoperatively and was debrided by shave excision technique under deep hypothermic circulatory arrest. Follow-up showed normal function of the tricuspid valve and preserved biventricular function.

Phrenic nerve injury after paediatric heart surgery: is aggressive plication of the diaphragm beneficial?

OBJECTIVES: Phrenic nerve injury after paediatric heart surgery is associated with significant morbidity. Surgical plication of the diaphragm is believed to be beneficial to the patient, with difficult weaning from ventilation; however, the optimal timing remains unclear. We aimed to compare the outcome after two different strategies for treating hemidiaphragmatic paresis. METHODS: A retrospective analysis of all patients with paresis of the diaphragm between 2000 and 2010 was performed, with special attention to the rate of reintubations, ventilation and intensive care unit (ICU) stay and the rate of plication. In 2005, the strategy for treating diaphragmatic paresis in our institution changed from conservative treatment with plication after multiple extubation efforts towards an aggressive one with plication after a single unsuccessful extubation. We compared the outcome of all patients and that of the newborns separately from the two periods. RESULTS: During the study period, 148 patients with diaphragmatic paresis were diagnosed and included. Median age at the cardiac operation was 7 months (1 day-18 years), ventilation time ranged from 4 h to 41 days (median 7 days), 42 (28.4%) of the patients required at least one reintubation and ICU stay ranged from 2 to 63 days (median 11 days). A total of 63 plications were performed-5 (9%) before 2004 and 58 (62%) after 2005, P < 0.001. There were no significant differences in the ventilation time-6 (1-40) vs 8 (0-41) days, P = 0.36, reintubation rate-28 vs 29%, P = 0.85 and ICU stay-10 (3-63) vs 12 (2-55) days, P = 0.41 between both groups. The newborn patients, treated with the different strategies, also did not differ significantly in their outcome: ventilation time-12 (2-40) vs 11.5 (3-34) days, P = 0.38; reintubation rate-43 vs 41%, P = 0.62; ICU stay-16 (6-63) vs 15 (7-55) days, P = 0.55. CONCLUSIONS: Changing the strategy for phrenic nerve injury after paediatric heart surgery towards a more aggressive one with early plication of the diaphragm was not associated with the better outcome. Prospective randomized studies are needed to determine the optimal management of this complication.

Inhaled nitric oxide for elevated cavopulmonary pressure and hypoxemia after cavopulmonary operations.

Background. Elevated cavopulmonary pressure early after surgical creation of cavopulmonary connections is an important hemodynamic problem with grave prognostic significance. We examined the effect of administration of inhaled nitric oxide (iNO) to patients with elevated cavopulmonary pressure in the early postoperative period. Methods. We retrospectively reviewed data pertaining to all 14 patients with superior (n = 6) and total (n = 8) cavopulmonary connections who were treated with iNO in the early postoperative period during an interval of six years. Changes in the cavopulmonary pressure, the transpulmonary gradient, and the oxygen saturation after the institution of iNO were evaluated. The preoperative characteristics of the patients were compared to those of a control group of patients with cavopulmonary operations not treated with iNO postoperatively. Results. Twelve hours after the initiation of iNO therapy, significant reduction in the cavopulmonary pressure (16.6 ± 3.5 mm Hg vs 18.1 ± 2.3 mm Hg, P = .006), reduction in the cavopulmonary gradient (7.0 ± 3.5 mm Hg vs 9.8 ± 3.7 mm Hg, P = .009), and elevation of the arterial oxygen saturation (84.5% ± 6.0% vs 78.7% ± 5.9%, P = .001) were observed. Linear correlation analysis confirmed tendencies for reduction in the cavopulmonary pressure (P = .13), reduction in the cavopulmonary gradient (P = .02), and elevation of the oxygen saturation (P = .10). Compared to the control group, the treated patients tended to have higher preoperative pulmonary arterial pressures (PAPs) 17 (11-30) mm Hg versus 12 (10-25) mm Hg, P = .10; higher pulmonary vascular resistance (PVR) 2.04 (0.27-6.94) Wood units versus 1.02 (0.49-5.20) Wood units, P = .37; and longer bypass times 154 (41-218) versus 91 (15-276) minutes, P = .13. Conclusions. Administration of iNO was associated with diminuition of cavopulmonary pressure and transpulmonary gradient and increasing oxygen saturation in our small group of patients. In our experience selected patients with preoperatively elevated PAP above 17 mm and PVR above 2 Wood units can undergo cavopulmonary operations with iNO treatment early postoperatively.

Left ventricular and mitral valve function long after repair of left anomalous coronary artery from the pulmonary artery: recovery years after severe ischemia.

BACKGROUND: The study evaluates the long-term results of surgery for anomalous left coronary artery from the pulmonary artery (ALCAPA) with special attention on the left ventricular (LV) function and mitral regurgitation. METHODS: Twenty-one children underwent surgery for ALCAPA over 23 years (1987-2010). All patients underwent establishment of a two-coronary system, by direct reimplantation (n = 13) or by intrapulmonary tunnel technique (n = 8), with concomitant mitral valve repair in one. The follow-up echocardiograms were evaluated to assess LV function and mitral regurgitation. RESULTS: Five patients died. The age of the nonsurvivors was lower, 4.2 ± 1.3 versus 22.7 ± 29.4 months, P = .04. All nonsurvivors had moderate or severe mitral regurgitation preoperatively and higher LV diameter z score than the survivors: 11.8 (9-14.6) versus 4.6 (1.9-13.1), P = .01. At last follow-up, all survivors were asymptomatic; the diastolic LV diameter was normal, with z scores: 0.3 (0.1-1.9) versus 7 (1.9-14.6) preoperatively, P = .001, as was the LV ejection fraction: 66% (61%-78%) versus 38% (16%-70%) preoperatively, P = .001. Fifteen patients had moderate or severe mitral regurgitation at initial presentation and it eventually regressed to insignificant in all survivors (P = .001). No subsequent interventions on the coronary arteries or the mitral valve were needed. Four patients with intrapulmonary tunnel had mild suprapulmonary obstruction with Doppler peak gradients between 20 and 30 mm Hg. CONCLUSIONS: In our experience, establishment of a two-coronary circulation without mitral valve repair leads to normalization of LV dimension and systolic function and to improvement of mitral regurgitation in the surviving patients. Mortality is related to low age and to the associated higher degree of LV dysfunction.