Acute onset of ornithine transcarbamylase deficiency after total anomalous pulmonary venous connection repair to a 2-day-old neonate.

Ornithine transcarbamylase deficiency is an X-linked disorder which results in the accumulation of ammonia causing irritability and vomiting. Acute hyperammonemia requires rapid and intensive intervention. However, as those clinical features are non-specific and commonly seen in peri-operative situation, ornithine transcarbamylase deficiency could be difficult to diagnose prior to and post-emergency cardiac surgery. We report a 2-day-old male neonate who was diagnosed with ornithine transcarbamylase deficiency presenting hyperammonemia and severe heart failure after total anomalous pulmonary venous connection repair.

[Right Atrial Blood Cyst with Patent Foramen Ovale:Report of a Case].

A woman in 70s was diagnosed with lung cancer, and a right atrial mass was discovered incidentally during preoperative examination by contrast-enhanced computed tomography (CT). Transesophageal echocardiography revealed a 20-mm, stemmed, spherical mass with low internal echogenicity and partially high echogenicity extending from the junction of the inferior vena cava to the posterior wall of the right atrium. Patent foramen ovale( PFO) was also confirmed. To avoid embolization and obtain diagnosis, the patient was referred for right atrial tumor resection. Cardiopulmonary bypass was established; the right atrial tumor was removed while the patient was in cardiac arrest. The tumor membrane was thin and easily ruptured, revealing jelly-like blood content and calcified mass. The patient recovered well after surgery and was discharged on day 15. According to the pathological examination, the tumor was a blood cyst. This is an extremely rare case of a blood cyst with PFO.

A valuable echocardiographic indicator for the optimal tightness of bilateral pulmonary artery banding.

OBJECTIVES: The optimal tightness of bilateral pulmonary artery banding (BPAB) is considered to balance not only systemic-to-pulmonary blood flow but also each pulmonary blood flow, which is still challenging. To achieve them, we adopt the end-diastolic velocity (EDV) to the peak systolic velocity (PSV) ratio at BPAB with intraoperative epicardial echocardiography. We evaluated the usefulness of the EDV to PSV ratio and the patient outcomes. METHODS: 34 patients underwent BPAB with this indicator and using a looped polytetrafluoroethylene suture. The PSV and the EDV to PSV ratio with echocardiography were measured in the intraoperative, early postoperative and late postoperative period. Lung perfusion scintigraphy was performed to quantify flow to each lung. RESULTS: There were 3 early deaths (< 30 days). Two patients required re-BPAB due to hypoxia. The intraoperative EDV to PSV ratios in the right and left were almost equal (0.50 ± 0.07 versus 0.51 ± 0.06, P = 0.73). There was no significant difference in the right and left EDV to PSV ratios throughout the postoperative course. The right PSV was smaller than the left PSV due to the Doppler angle intraoperatively (2.78 ± 0.57 versus 3.02 ± 0.50, P = 0.030). In addition, the PSV changed significantly until the late postoperative period (P < 0.001). Lung perfusion scintigraphy revealed only two patients had perfusion abnormalities. CONCLUSIONS: Our clinical outcomes are satisfactory with low early mortality and a low rate of re-BPAB. The EDV to PSV ratio can be a reliable indicator to assess flow distribution to each lung and may be a valuable adjunct to achieve balanced systemic to pulmonary flow.

Surgical outcomes of reoperation after Fontan completion.

OBJECTIVES: Patients who have achieved Fontan circulation may require reoperation. We reviewed the outcomes of reoperation after Fontan completion and assessed the risk factors for poor outcomes. METHODS: This was a retrospective study of 106 patients undergoing open-heart reoperations after Fontan completion in 2003 at a single institution. RESULTS: The mean age at reoperation was 24.6 ± 8.3 years. A history of Fontan failure or end-organ dysfunction was noted in 30 patients. The reoperations included 73 total cavopulmonary connection conversions, 29 atrioventricular or semilunar valve operations (17 with total cavopulmonary connection conversions) and 4 other operations. Eight early deaths occurred. During a median follow-up of 5.5 (0.01-16.2) years, there were 3 late deaths and 9 second cardiac operations. The 10-year survival rate after reoperation was 89.8%, and the 5-year second cardiac operation-free survival was 84.3%. The 10-year survival rates were significantly lower in patients who underwent surgery before 2011 (75.8% vs 100%), had a history of Fontan failure or end-organ dysfunction (71.7% vs 97.3%), had preoperative central venous pressure >15 mmHg (64.9% vs 96.5%) and were operated on with deep hypothermic circulatory arrest (DHCA) (60.0% vs 91.3%). A history of Fontan failure or end-organ dysfunction, preoperative central venous pressure >15 mmHg and requirement of DHCA were identified as risk factors for mortality. CONCLUSIONS: Reoperation after Fontan completion resulted in excellent mid-term outcomes. A history of failed Fontan circulation and the requirement of DHCA negatively affected survival outcomes.

Modified double patch repair with infarct exclusion technique for ventricular septal perforation: a case study.

BACKGROUND: Ventricular septal perforation (VSP) after acute myocardial infarction (AMI) is accompanied by the worsening of rapid hemodynamics, resulting in a poor prognosis. In our department, infarct lesions are preoperatively detected with electrocardiogram (ECG)-synchronized contrast computed tomography, and the scope of approach and exclusion is determined. Furthermore, to effectively prevent a residual shunt, modified double patch repair and infarct exclusion techniques were used in combination to preserve left ventricular (LV) function. This method is reported because it considers both techniques as a surgical procedure that can be accomplished relatively easily and simultaneously. CASE PRESENTATION: We targeted two consecutive VSP patients who underwent this procedure. It took an average of 1 day from the onset of VSP to surgery. We performed double patch and infarct exclusion for VSP using bovine pericardium via an LV incision. Two patches were marked with a skin pen to anastomose eight mattresses equally. In addition, a one piece-coupled patch was made for infarct exclusion. The two patients were extubated on the day after surgery and intra-aortic balloon pump assistance was also withdrawn. Without perioperative complications, they could leave the intensive care unit after 6.5 days on average. Early postoperative ECG and magnetic resonance angiography showed good LV wall contraction, except at the infarcted area, with no evidence of a residual shunt. CONCLUSION: The modified double patch repair with infarct exclusion technique is more effective for preventing a residual shunt and maintaining postoperative cardiac function than either of the techniques alone.

Long-term outcomes of total correction for isolated total anomalous pulmonary venous connection: lessons from 50-years' experience.

OBJECTIVES: Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity. METHODS: A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach. RESULTS: There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight <2 kg and with postoperative PVO (P < 0.0001, P = 0.0041, P = 0.0003, respectively). Reoperations were performed 27 times in 22 patients (PVO repair, 11; staged repair, 4 and others, 12). PVO repair was performed at a mean of 2.5 ± 1.6 months later. The actuarial freedom from reoperation rates were 88.8% and 83.2% at 20 and 30 years, respectively. Multivariable analysis revealed that the risk of reoperation was associated with mixed-type TAPVC and postoperative PVO (P = 0.0064 and P < 0.0001, respectively). CONCLUSIONS: Long-term surgical outcomes of isolated TAPVC have improved over the past 25 years. Postoperative PVO, the mixed-type TAPVC and a body weight <2 kg might be the important factors contributing to mortality and morbidity.