Improvement of lung function and pulmonary hypertension after pulmonary aneurysm repair: case series

Pulmonary artery aneurysms (PAA) can be complicated with pulmonary arterial hypertension (PAH), causing sudden death due to PA rupture and dissection. Because treatment with PAH-targeted drugs does not always prevent PAA progression, prophylactic surgical repair of the PAA seems a promising alternative. However, although it avoids rupture and dissection of the PAs, additional benefits have not been forthcoming. We therefore present two patients with co-existing PAH and a PAA who underwent surgical repair of the aneurysm. Following the surgery, their lung function and pulmonary hypertension improved. Optimal treatment of PAA remains uncertain, however, with no clear guidelines regarding the best therapeutic approach. This case series provides physicians with reasons to repair PAA surgically in patients with PAH

Intravenous infusion of cardiac progenitor cells in animal models of single ventricular physiology

OBJECTIVES: The goal of this study was to identify the practical applications of intravenous cell therapy for single-ventricle physiology (SVP) by establishing experimental SVP models. METHODS: An SVP with a three-stage palliation was constructed in an acute swine model without cardiopulmonary bypass. A modified Blalock–Taussig (MBT) shunt was created using an aortopulmonary shunt with the superior and inferior venae cavae (SVC and IVC, respectively) connected to the left atrium (n = 10). A bidirectional cavopulmonary shunt (BCPS) was constructed using a graft between the IVC and the left atrium with an SVC cavopulmonary connection (n = 10). The SVC and the IVC were connected to the pulmonary artery to establish a total cavopulmonary connection (TCPC, n = 10). The survival times of half of the animal models were studied. The other half and the biventricular sham control (n = 5) were injected intravenously with cardiosphere-derived cells (CDCs), and the cardiac retention of CDCs was assessed after 2 h. RESULTS: All SVP models died within 20 h. Perioperative mortality was higher in the BCPS group because of lower oxygen saturation (P  CONCLUSIONS: Without the total right heart exclusion, stage-specific SVP models can be functionally constructed in pigs with stable outcomes. Intravenous CDC injections may be applicable in patients with SVP before TCPC completion, given that the initial lung trafficking is efficiently bypassed and sufficient systemic blood flow is supplied from the single ventricle

Anatomical Risk Factors for Reintervention after Arterial Switch Operation for Taussig–Bing Anomaly

Background: This study aimed to determine the factors related to reintervention, especially for pulmonary artery stenosis (PS), in patients with Taussig–Bing anomaly (TBA) after arterial switch operation (ASO). Methods:This retrospective study included 34 patients with TBA who underwent ASO between 1993 and 2018. Preoperative anatomical and physiological differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries (TGA) with ventricular septal defect (VSD) and TBA with an anterior and rightward aorta. Results: The median age and body weight at ASO were 43 (16–102) days and 3.6 (2.8–3.8) kg, respectively. Aortic arch obstruction and coronary anomalies were present in 64% and 41% patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery: aortic annulus size ratio than those not receiving reintervention (1.69 vs. 1.41, P = 0.02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the TGA/VSD group. PS-related reintervention was required more in the TBA group than in the TGA/VSD group. Conclusions: Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomical relationship during ASO. (243 words

Successful intra-postoperative extracorporeal circulatory support with atrial communication for treatment of anomalous left coronary artery from the pulmonary artery : a case report

We describe a successful case of surgical treatment for anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome with severe left ventricular dysfunction. Because of the severe left ventricular dysfunction, we planned to use an extracorporeal membrane oxygenation for heart support until a satisfactory recovery had been established. The left ventricular function signifi cantly recovered in a few days, and the patient could be discharged without any complications.</p

Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes

Background This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot (TOF) on pulmonary valve annulus (PVA) growth, the rate of valve-sparing repair (VSR) at the time of intracardiac repair (ICR), and long-term functional outcomes. Methods This retrospective study included 330 patients with TOF who underwent ICR between 1991 and 2019, including 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0±7.3 years. We compared the data of patients who underwent BTS and those who did not undergo BTS before ICR. Results The median age and body weight before BTS were 71 (28–199) days and 4.3 (3.3–6.8) kg respectively. There were no in-hospital or interstage deaths after BTS. The PVA Z-scores of patients with BTS revealed significant growth after BTS (from -4.2±1.8 to -3.0±1.7, P Conclusions A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. BTS may have contributed to the avoidance of primary transannular patch repair (TAP) and facilitated PVA growth; therefore, approximately half of the symptomatic neonates and infants were recruited for VSR. Staged repair may have led to functionally-reliable delayed TAP repair, thereby resulting in less surgical reinterventions

Norwood procedure with right ventricle to pulmonary artery conduit: a single-centre 20-year experience

Objectives: The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex (HLHC). Methods: A retrospective observational study was performed in 136 patients with HLHC who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analyzed. Results: Stage 1 survival was 91.9% (125/136). Reintervention for pulmonary artery stenosis was needed for 22% and 30% at stage 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while four were not considered to be Fontan candidates. Risk factors for overall mortality included weighing Conclusions: Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain pulmonary artery growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion

The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa

ObjectiveTricuspid valve size is the major determinant of outcomes for patients with pulmonary atresia with intact ventricular septum. Lack of right ventricle–pulmonary artery continuity is associated with poor tricuspid valve growth (decrement in Z-value). However, most reports did not show evidence for disproportionate growth of the tricuspid valve after establishment of right ventricle–pulmonary artery continuity.MethodsWe studied 40 patients with pulmonary atresia with intact ventricular septum who underwent initial right ventricular decompression for planned staged repair. The initial Z-value of the tricuspid valve diameter (Zt1) was obtained from the echocardiography-derived normal value. The late Z-value (Zt2) was measured before definitive repair or the last available Z-value, if definitive repair was not yet reached. The factors associated with the changes of Z-values (Zt2 − Zt1) were analyzed.ResultsThe mean initial tricuspid Z-value (Zt1) was −6.2 ± 3.5. After treatment (Zt2), the mean Z-value was −6.0 ± 3.4 (n = 34). Overall, the tricuspid Z-values did not change. Individually, the change in Z-value (Zt2 − Zt1) was larger than +2 in 11 (32%) patients and smaller than −2 in 6 (18%) patients. Increases in Z-value (Zt2 − Zt1) were significantly associated with right ventricular pressure/left ventricular pressure ratio measured after initial palliation (r = −0.54; P = .001) and the initial tricuspid valve Z-value (Zt1) (r = −0.40; P = .02).ConclusionsDisproportional growth of the tricuspid valve can occur, especially in patients with small tricuspid valves and lower right ventricular pressures after decompression. The findings support the possibility of neonates with small tricuspid valves undergoing biventricular repair after right ventricular decompression surgery

Extracorporeal membrane oxygenation following pediatric cardiac surgery: development and outcomes from a single-center experience

　Extracorporeal membrane oxygenation (ECMO) has emerged as an effective mechanical support following cardiac surgery with respiratory and cardiac failure. However, there are no clear indications for ECMO use after pediatric cardiac surgery. We retrospectively reviewed medical records of 76 pediatric patients [mean age, 10.8 months (0-86); mean weight, 5.16 kg (1.16-16.5)] with congenital heart disease who received ECMO following cardiac surgery between January 1997 and October 2010. Forty-five patients were treated with an aggressive ECMO approach (aggressive ECMO group, April 2005-October 2010) and 31 with a delayed ECMO approach (delayed ECMO group, January 1997-March 2005). Demographics, diagnosis, operative variables, ECMO indication, and duration of survivors and non-survivors were compared. Thirty-four patients (75.5%) were successfully weaned from ECMO in the aggressive ECMO group and 26 (57.7%) were discharged. Conversely, eight patients (25.8%) were successfully weaned from ECMO in the delayed ECMO group and two (6.5%) were discharged. Forty-five patients with shunted single ventricle physiology (aggressive: 29 patients, delayed: 16 patients) received ECMO, but only 15 (33.3%) survived and were discharged. The survival rate of the aggressive ECMO group was significantly better when compared with the delayed ECMO group (p<0.01). Also, ECMO duration was significantly shorter among the aggressive ECMO group survivors (96.5 ± 62.9 h, p<0.01). Thus, the aggressive ECMO approach is a superior strategy compared to the delayed ECMO approach in pediatric cardiac patients. The aggressive ECMO approach improved our outcomes of neonatal and pediatric ECMO