Right Ventricle-to-Pulmonary Artery Shunt in Pulmonary Atresia with a Ventricular Septal Defect: A Word of Caution.

The management of pulmonary atresia with a ventricular septal defect (PA/VSD) depends on the anatomy of the pulmonary artery or on the surgical strategy used at individual institutions. In our institution, we adopted a right ventricle-to-pulmonary artery (RV-PA) shunt in 2011 as a palliative procedure for PA/VSD to overcome the disadvantages of a Blalock-Taussig shunt. We evaluated the results of the RV-PA shunt as initial palliative surgery for PA/VSD. Thirteen patients with ductus-dependent PA/VSD from August 2011 to August 2015 were enrolled. The mean age at surgery was 17.9 ± 15.3 (range 5-60) days, and the mean body weight was 2.9 ± 0.6 (range 2.2-4.0) kg. A Gore-Tex tube graft was used in all patients. We retrospectively observed intra- and postoperative complications, early and late mortality, and palliation duration to definitive repair. Left pulmonary artery angioplasty was performed as a concomitant procedure in three patients. There were no early hospital mortalities, although two inter-stage deaths occurred 34 and 47 days postoperatively: one patient died of aspiration and the other of right ventricular outflow tract (RVOT) pseudoaneurysm rupture. Two patients (15.4%) required the extracorporeal membrane oxygenation support postoperatively: one because of failure to wean from a bypass caused by persistent hypoxemia and the other because of sudden massive bleeding from the RVOT suture line in the intensive care unit 2 days postoperatively. These two patients underwent second-stage definitive repair successfully. Five patients (41.7%) required catheter intervention, for juxtaductal left pulmonary artery stenosis in three patients, right pulmonary artery stenosis in one, and shunt inflow stenosis in one. Two patients (15.4%) required re-operation because of shunt inflow stenosis and RVOT pseudoaneurysm, respectively. All patients who survived the RV-PA shunt underwent total correction at a mean interval of 13.1 months. A RV-PA shunt is an option for the initial palliation of ductus-dependent PA/VSD. Major complications can occur, including RVOT pseudoaneurysm, shunt inflow stenosis, persistent hypoxemia during the immediate postoperative period, and dehiscence of the shunt anastomosis site. Caution should be taken when performing the RV-PA shunt for palliation of PA/VSD.

Branch pulmonary artery peel operation in a patient without a native intrapericardial pulmonary artery.

A patient with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries without an intrapericardial pulmonary artery (PA) underwent a one-stage total correction, including both branch PA reconstructions, with a bovine pericardial roll at the age of 42 months. She was readmitted 54 months after the operation because of extracardiac conduit bacterial endocarditis and pulmonary infarction. The bovine pericardial roll between the right and left PAs was enucleated, and the surrounding fibrotic tissue (peel) was used as a new branch PA vascular conduit. A cardiac catheterization performed 86 months after the peel operation showed no aneurysmal dilatation, stenosis, or thrombotic occlusion.

Effects of Balloon Pulmonary Valvuloplasty as Preoperative Palliation for Tetralogy of Fallot.

OBJECTIVES: We investigated the effect of balloon pulmonary valvuloplasty (BPV) as a palliative procedure for patients with tetralogy of Fallot (TOF). METHODS: This was a retrospective single-center study conducted between 2008 and 2014. We classified patients into three groups according to palliation: treatment with BPV (group I), with a Blalock-Taussig shunt (group II), and with infundibulectomy (group III). The growth of the pulmonary valve (PV) annulus and need for transannular patching (TAP) during total correction were compared between groups. RESULTS: Forty-eight patients were enrolled: 31, 10, and 7 in groups I, II, and III, respectively. The mean ages at palliation were 26.3 ± 23.6, 21.5 ± 16.4, and 15.2 ± 4.2 days in groups I, II, and III, respectively (P = .867). The success rate of BPV was 90.3%. The initial PV z-score before palliation did not differ between groups I, II, and III (-3.14 ± 1.44, -3.84 ± 1.47, and -3.71 ± 1.68; P = .494); the preoperative PV z-score before total correction was larger in group I than in group II (-1.19 ± 1.14 and -3.05 ± 1.19; P = .001), and also larger in group III than in group II (group III: -1.10 ± 0.90; P = .003). The change in pulse oxygen saturation differed significantly between the three groups (P = .031), particularly between groups I and II (P = .010), and the change in PV z-score was greater in groups I and III than in group II (P < .001). TAP during total correction was performed in 29.0%, 90.0%, and 16.7% of groups I, II, and III, respectively (P = .001; group I vs. II, P = .001; group II vs. III, P = .008). CONCLUSION: BPV was an effective and safe palliation that increased pulmonary blood flow and promoted growth of the PV annulus in selected symptomatic newborns with TOF.

Acute tubular necrosis as a part of vancomycin induced drug rash with eosinophilia and systemic symptoms syndrome with coincident postinfectious glomerulonephritis.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal condition characterized by skin rash, fever, eosinophilia, and multiorgan involvement. Various drugs may be associated with this syndrome including carbamazepine, allopurinol, and sulfasalazine. Renal involvement in DRESS syndrome most commonly presents as acute kidney injury due to interstitial nephritis. An 11-year-old boy was referred to the Children's Hospital of Pusan National University because of persistent fever, rash, abdominal distension, generalized edema, lymphadenopathy, and eosinophilia. He previously received vancomycin and ceftriaxone for 10 days at another hospital. He developed acute kidney injury with nephrotic range proteinuria and hypocomplementemia. A subsequent renal biopsy indicated the presence of acute tubular necrosis (ATN) and late exudative phase of postinfectious glomerulonephritis (PIGN). Systemic symptoms and renal function improved with corticosteroid therapy after the discontinuation of vancomycin. Here, we describe a biopsy-proven case of severe ATN that manifested as a part of vancomycin-induced DRESS syndrome with coincident PIGN. It is important for clinicians to be aware of this syndrome due to its severity and potentially fatal nature.

Percutaneous closure of pseudoaneurysm in the left ventricle in a young child.

Pseudoaneurysm in the left ventricular outflow tract is a rare complication occurring after cardiac surgery. We report on the successful percutaneous device closure of a pseudoaneurysm in the left ventricular outflow tract, which developed in a 23-month-old girl after resection of a subaortic ridge. The pseudoaneurysm was closed using an Amplatzer Vascular Plug II.

A Novel Predictive Value for the Transannular Patch Enlargement in Repair of Tetralogy of Fallot.

BACKGROUND: In tetralogy of Fallot, whether relieving right ventricular outflow tract obstruction requires transannular patch enlargement (TAPE) of the pulmonary valve depends on pulmonary valvular annulus size. The z-score of pulmonary annulus is most commonly used as a predictor of the need for TAPE. However, the z-score is a complex value affected by height, body weight, body surface area, and different reference populations. Therefore, we hypothesized that the great artery annulus size ratio (pulmonary valvular annulus size to aortic valve annuls size [GA ratio]) may be a better predictor of the need for TAPE. METHODS: We analyzed 122 patients retrospectively who had undergone total correction of tetralogy of Fallot between January 2007 and March 2015. We categorized the patients into two groups, TAPE versus non-TAPE. Great arterial annuli sizes were evaluated in each group, and the GA ratio cutoff values for TAPE were calculated. RESULTS: In total, 40 patients (32.8%) required TAPE. Both GA ratios and z-scores were smaller in the TAPE group than in the non-TAPE group (0.51 versus 0.67, p < 0.0001, and -2.46 versus -0.85, p < 0.0001, respectively). In receiver operating characteristics analyses, the z-score and GA ratio cutoff values were -1.67 (area under the curve = 0.797) and 0.56 (area under the curve = 0.900), respectively, demonstrating that the GA ratio was a more powerful diagnostic tool as a predictor of TAPE (p = 0.014). CONCLUSIONS: Our results suggest that the GA ratio is a useful predictor for TAPE and can be applied readily and simply in clinical practice.

Sutureless Patch Angioplasty for Postoperative Pulmonary Artery Stenosis in Congenital Cardiac Surgeries.

BACKGROUND: Reconstruction of branch pulmonary arteries (PAs) can be demanding in redo congenital cardiac surgeries. Sutureless patch angioplasty could be a useful method to solve this problem, and we evaluated the feasibility of sutureless patch angioplasty for postoperative PA stenosis in patients who underwent congenital cardiac surgery. METHODS: We retrospectively reviewed 28 patients (19 males and 9 females) who underwent sutureless patch angioplasty for postoperative PA stenosis between November 2004 and April 2015. The median age was 7.3 months (range, 4.3 to 54.7), and the median weight was 7.2 kg (range, 5.3 to 12.2 kg). Right PA angioplasty was performed in 5, left PA angioplasty in 10, and both sides in 13 patients. The most common original diagnosis was hypoplastic left heart syndrome (n = 18 of 28, 64.3%). Concomitant surgeries were the bidirectional cavopulmonary shunt in 24 patients, the Fontan operation in 3, and the Rastelli operation in 1 patient. RESULTS: No operative death occurred. However, 2 late deaths were recorded, and both were unrelated to PA angioplasty. Mean follow-up duration was 60.9 ± 33.1 months. None of the patients had postoperative bleeding or thrombotic occlusion. No reoperation for PA restenosis was performed, and only 1 patient (3.6%) had a PA balloon angioplasty with a good result 12.9 months after the operation. Echocardiography or computed tomography angiography at the recent follow-up showed good branch PAs in all patients. CONCLUSIONS: Sutureless patch angioplasty for postoperative PA stenosis could simplify PA angioplasty, and be a safe and effective method for PA reconstruction in patients who undergo congenital cardiac surgery.

A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect.

OBJECTIVES: We evaluated our clinical experiences on rehabilitation of native pulmonary arteries (PAs) with a central shunt using an expanded polytetrafluoroethylene (ePTFE) tube graft in management of pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) with diminutive PAs. METHODS: From March 1993 to June 2013, a total of 28 consecutive patients underwent surgery for pulmonary atresia with VSD and MAPCAs. We identified 15 patients who underwent a central shunt procedure using an ePTFE tube graft. Median age and weight at the initial operation were 1.91 months and 5.22 kg, respectively. Ligation of MAPCAs was performed in 3 patients concomitantly. The techniques used were a U-shaped central shunt in 13 patients, and a side-to-side aorto-ePTFE tube graft anastomosis in 2 patients. RESULTS: There was no surgical mortality, and no shunt occlusion. The mean follow-up duration was 70.7 ± 67.1 months. Complete repair was achieved in 13 patients (13 of 14 of the follow-up patients; 92.9%), and the remaining patient is waiting for complete repair. The median age at complete repair was 19.4 months. The mean PA index before the initial central shunt procedure was 22.7 ± 13.2 mm(2)/m(2), which increased to 149.4 ± 86.6 mm(2)/m(2) at the final recordings before shunt division, and 185.9 ± 84.0 mm(2)/m(2) before complete repair. There were 2 late mortality cases. The overall survival rates were 92.9%, 82.5%, and 82.5%, at 1, 5, and 10 years, respectively. CONCLUSIONS: A central shunt, using an ePTFE tube graft, is an effective initial palliative procedure for rehabilitation of the diminutive PAs in patients with pulmonary atresia with VSD and MAPCAs.