Virtual treatment planning in three patients with univentricular physiology using computational fluid dynamics-Pitfalls and strategies.

Background: Uneven hepatic venous blood flow distribution (HFD) to the pulmonary arteries is hypothesized to be responsible for the development of intrapulmonary arteriovenous malformations (PAVM) in patients with univentricular physiology. Thus, achieving uniform distribution of hepatic blood flow is considered favorable. However, no established method for the prediction of the post-interventional hemodynamics currently exists. Computational fluid dynamics (CFD) offers the possibility to quantify HFD in patient-specific anatomies before and after virtual treatment. In this study, we evaluated the potential benefit of CFD-assisted treatment planning. Materials and methods: Three patients with total cavopulmonary connection (TCPC) and PAVM underwent cardiovascular magnetic resonance imaging (CMR) and computed tomography imaging (CT). Based on this imaging data, the patient-specific anatomy was reconstructed. These patients were considered for surgery or catheter-based intervention aiming at hepatic blood flow re-routing. CFD simulations were then performed for the untreated state as well as for different surgical and interventional treatment options. These treatment options were applied as suggested by treating cardiologists and congenital heart surgeons with longstanding experience in interventional and surgical treatment of patients with univentricular physiology. HFD was quantified for all simulations to identify the most viable treatment decision regarding redistribution of hepatic blood flow. Results: For all three patients, the complex TCPC anatomy could be reconstructed. However, due to the presence of metallic stent implants, hybrid models generated from CT as well as CMR data were required. Numerical simulation of pre-interventional HFD agreed well with angiographic assessment and physiologic considerations. One treatment option resulting in improvement of HFD was identified for each patient. In one patient follow-up data after treatment was available. Here, the virtual treatment simulation and the CMR flow measurements differed by 15%. Conclusion: The combination of modern computational methods as well as imaging methods for assessment of patient-specific anatomy and flow might allow to optimize patient-specific therapy planning in patients with pronounced hepatic flow mismatch and PAVM. In this study, we demonstrate that these methods can also be applied in patients with complex univentricular physiology and extensive prior interventions. However, in those cases, hybrid approaches utilizing information of different image modalities may be required.

A Successful Repair of Pseudocoarctation With Associated Aortic Arch Aneurysm in an Eight-Month-Old Infant.

Pseudocoarctation of the aorta is an uncommon congenital entity characterized by juxtaductal elongation and tortuosity of the aortic arch. It is known to be associated with aneurysm formation. Our patient is a 7.7-month-female infant followed prenatally by magnetic resonance imaging that detected this anomaly. Subsequently, postnatal preoperative computed tomography angiography was confirmatory. The operation comprised resection of the aneurysmal portion of the aorta and extended end-to-end reconstruction, without prosthesis usage. The left subclavian artery was reimplanted into the left carotid artery. The postoperative course was uneventful. Computed tomography aortography and transthoracic echocardiography demonstrated no residual stenosis at six months of follow-up.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.

Transesophageal Echocardiographic Predictor of Significant Right Ventricular Outflow Tract Obstruction After Tetralogy of Fallot Repair.

The optimal method for reconstruction of the right ventricular outflow tract in tetralogy of Fallot repair is yet to be established. Transatrial repair with pulmonary valve preservation avoids the detrimental effects of ventriculotomy and transannular patch; however, because of moderately hypoplastic pulmonary valve, these techniques are associated with undesirable residual right ventricular outflow tract gradients resulting in reoperations. The study sought to define intraoperative echocardiographic predictors of significant right ventricular outflow tract obstruction after tetralogy of Fallot repair. From July 2016 to December 2017, 58 patients with tetralogy of Fallot underwent repair with direct intraoperative pressures measuring and intraoperative transesophageal echocardiography performing after tetralogy of Fallot repair completion. Multivariate analysis was performed using logistic regression analysis to identify independent predictors of right ventricular outflow tract obstruction. One year after tetralogy of Fallot repair, significant right ventricular outflow tract obstruction was present in 12 patients (20.6%): 10 patients (26.2%) after pulmonary valve-sparing procedure and 2 patients (12.5%) after transannular patch (P = 0.35). Right ventricular outflow tract z-score lower than -3.2 had the best predictive value for significant residual right ventricular outflow tract obstruction (area under the curve = 0.979, P < 0.001). Multivariate analysis found that the right ventricular outflow tract z-score reached a statistical significance as a predictor of significant right ventricular outflow tract obstruction (P < 0.0001). Right ventricular outflow tract z-score <-3.2 measured by intraoperative transesophageal echocardiography is an indicator of postoperative right ventricular outflow tract obstruction right after surgery and a predictor of obstruction development following tetralogy of Fallot repair.

Single-centre 20-year experience with repair of truncus arteriosus.

OBJECTIVES: We analysed a large series of truncus arteriosus repairs with a focus on early and late outcomes. METHODS: Ninety-seven consecutive patients who underwent truncus arteriosus repair (1997-2017) were included retrospectively. Univariable analysis for mortality and reintervention was performed. RESULTS: The early mortality rate decreased from 45% (1997-2007; 14/31) to 4.5% (2008-2017; 3/66) (P = 0.001). Repair beyond the neonatal period (P = 0.03) and direct connection for right ventricular outflow tract reconstruction (P = 0.001) were associated with early death by univariable analysis. Overall survival was 68 ± 6.0% at 15 years; a majority of the deaths (90%; 9/10) occurred within the first year after repair. Freedom from the first and second conduit reoperations at 10 years was 22.9% and 89%, respectively. Freedom from truncal valve (TrV) reoperation was 83.9% at 15 years. Initial TrV insufficiency ≥ moderate was associated with a TrV reoperation (P = 0.008) with freedom from TrV reoperation in this subgroup of 58.3% at 10 years. Freedom from TrV reoperation for quadricuspid and tricuspid TrVs was 66.8% and 93.8% at 10 years with 100% for bicuspid TrVs at 8 years. At the last follow-up, 98.5% (69/70) were in New York Heart Association functional class I-II. CONCLUSIONS: In the current era, truncus arteriosus can be repaired with a low early mortality rate and a good long-term outcome. A significant reintervention burden still persists. Direct connection is associated with early mortality.

Ross-Konno Procedure With Cylinder Mitral Valve Replacement in 49 Days Old Infant.

A neonate with antenatally diagnosed severe aortic valve stenosis, left ventricular outflow tract obstruction, left ventricular endocardial fibroelastosis, and severe mixed mitral valve disease was admitted to our center for balloon aortic valvuloplasty. On day 49, we performed a Ross-Konno procedure and mitral valve replacement on the baby, whose weight was 3.4 kg. Right ventricle-to-pulmonary artery continuity was restored with a handmade trileaflet Gore-Tex conduit. We used a handmade cylindrical prosthesis made from decellularized equine pericardium in the mitral position. We observed improvement in left ventricular function and good performance of aortic, pulmonary, and mitral prostheses at four months of follow-up.

Five-year Experience With Arterial Switch Operation in the First Hours of Life.

We assessed morbidity after 2 management strategies for arterial switch operation (ASO) in a single institution: first hours of life surgery and conventional approach. From September 2009 to September 2014, 346 consecutive patients who underwent ASO were enrolled. The study group included 92 patients who underwent ASO in the first 24 hours after birth (group I). The control group consisted of 254 patients who underwent ASO after 24 hours of life in the conventional way (group II). Three outcomes were analyzed: postoperative ventilation time (VT), post-extubation hospital length of stay (peLOS), and a composite morbidity index, defined as postoperative VT + peLOS + occurrence of selected major complications. Overall 30-day survival was 98% (2 [2%] group I vs 5 [2%] group II, P = 1.000). Fifty (13.3%) major complications were observed: 14 (15%) in group I and 36 (15%) in group II (P = 0.635). Although peLOS and morbidity index did not differ significantly between groups, postoperative VT (42 hours [24, 67] vs 27 hours [22, 47], P = 0.008) and total hospital stay were significantly longer in group II (18 days [10, 19] vs 14 days [12, 18]). A median volume of 80 mL (60-100 mL) of autologous umbilical cord blood was collected (80 mL, group 1 vs 60 mL, group II; P = 0.090). Homologous blood cell transfusion was avoided in 70 patients (78%) in group I and in 13 patients (6%) in group II (P < 0.001). Arterial switch operation during the initial 24 hours of life has similar outcomes in view of morbidity and mortality to the conventional approach.

Surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries in 83 consecutive patients.

OBJECTIVES: This article reports the safety and efficacy of a morphology-based algorithm for the surgical management of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. METHODS: A total of 83 patients were operated from 2007 to 2014 using 3 surgical approaches: one-stage total repair, one-stage unifocalization with central shunt and delayed total repair and multistage unifocalization with subsequent total repair. Patients were divided into 2 groups depending on the surgical strategy used to choose the surgical approach. In Group I (2007-09), the surgeon-preferred procedure (71%-one-stage total repair) was used. In Group II (2010-14), a surgical algorithm based on pulmonary arterial tree morphology was applied. RESULTS: Median follow-up was 5.04 years and 98% complete. The overall survival rate was 92.59%. Cumulative operative mortality decreased from 10.7% in Group I to 0% in Group II ( P = 0.036). Successful complete repair was performed in 23 (82%) of 28 patients in Group I (median follow-up, 7.99 years) and in 33 (60%) of 55 patients in Group II (median follow-up, 3.85 years). The difference in total survival was not significant between Groups I and II but was markedly influenced by the presence of the 22q11 deletion. The survival of 23 patients with confirmed 22q11 deletion was 73.91%; the survival of 28 patients with confirmed absence of the 22q11 deletion was 100%; and the survival of 32 patients not tested for the 22q11 deletion was 100% ( P = 0.0001). CONCLUSIONS: Total survival was significantly lower in patients with the 22q11 deletion. Surgical management based on preoperative pulmonary arterial anatomical features improves early surgical results.

Simultaneous arterial switch and totally anomalous pulmonary venous connection repair in a 5-hour-old child, complicated by pulmonary venous stenosis.

A 5-hour-old boy underwent arterial switch operation and totally anomalous pulmonary venous connection repair. Subsequently developed severe pulmonary venous stenosis was successfully treated with hybrid balloon angioplasty.

Cerebral Thromboembolism From Ligated Blalock-Taussig Shunt in an Adult Patient.

A 26-year-old woman underwent tetralogy of Fallot repair and classic Blalock-Taussig shunt (BTS) ligation. On postoperative day 4 she developed severe left-sided hemiplegia due to cerebral infarction. Thrombi in the BTS stump, right brachiocephalic artery, and its branches were removed surgically and the BTS stump was resected. The patient gradually recovered from hemiplegia and is leading a normal life with moderate left hemiparesis and is in good functional status 4 years after the event. An aggressive approach in treating this complication was crucial to prevent further thrombosis and neurologic deficit progression in this patient. We recommend reducing the size of the BTS stump during definitive repair.

Anomalous origin of circumflex coronary artery from right pulmonary artery associated with coarctation of the aorta: a case report of surgical treatment.

Origin of the circumflex coronary artery (Cx) from the pulmonary arteries is an extremely rare anomaly. We describe a two-month-old female patient with anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta. Reimplantation of the anomalous Cx to the aorta and coarctation repair were performed. There were no postoperative complications, and the patient was discharged in satisfactory condition.