Switching the Left and the Right Hearts: A Novel Bi-ventricle Mechanical Support Strategy with Spared Native Single-Ventricle.

End-stage Fontan patients with single-ventricle (SV) circulation are often bridged-to-heart transplantation via mechanical circulatory support (MCS). Donor shortage and complexity of the SV physiology demand innovative MCS. In this paper, an out-of-the-box circulation concept, in which the left and right ventricles are switched with each other is introduced as a novel bi-ventricle MCS configuration for the "failing" Fontan patients. In the proposed configuration, the systemic circulation is maintained through a conventional mechanical ventricle assist device (VAD) while the venous circulation is delegated to the native SV. This approach spares the SV and puts it to a new use at the right-side providing the most-needed venous flow pulsatility to the failed Fontan circulation. To analyze its feasibility and performance, eight SV failure modes have been studied via an established multi-compartmental lumped parameter cardiovascular model (LPM). Here the LPM model is experimentally validated against the corresponding pulsatile mock-up flow loop measurements of a representative 15-year-old Fontan patient employing a clinically-approved VAD (Medtronic-HeartWare). The proposed surgical configuration maintained the healthy cardiac index (3-3.5 l/min/m2) and the normal mean systemic arterial pressure levels. For a failed SV with low ejection fraction (EF = 26%), representing a typical systemic Fontan failure, the proposed configuration enabled a ~ 28 mmHg amplitude in the venous/pulmonary waveforms and a 2 mmHg decrease in the central venous pressure (CVP) together with acceptable mean pulmonary artery pressures (17.5 mmHg). The pulmonary vascular resistance (PVR)-SV failure case provided a ~ 5 mmHg drop in the CVP, with venous/pulmonary pulsatility reaching to ~ 22 mmHg. For the high PVR failure case with a healthy SV (EF = 44%) pulmonary hypertension is likely to occur as expected. While this condition is routinely encountered during the heart transplantation and managed through pulmonary vasodilators a need for precise functional assessment of the spared failed-ventricle is recommended if utilized in the PVR failure mode. Comprehensive in vitro and in silico results encourage this novel concept as a low-cost, more physiological alternative to the conventional bi-ventricle MCS pending animal experiments.

Open Repair of a Complicated Late Endoleak Induced by Another Endoleak.

A 66-year-old man had an enlarging aortic aneurysm sac after an endovascular aortic replacement procedure that had been performed at another institution 4 years previously; it was without any endoleak but was complicated by the occlusion of the left limb, requiring cross-femoral bypass. Current computed tomography revealed dilatation of the proximal neck and the right common iliac artery. A type Ib endoleak was found from the distal end of the right limb of the endograft, possibly secondary to the dilatation of the artery around it; it then pressurized and caused the dilatation of the juxtarenal aorta around the proximal landing zone and induced a concomitant type Ia endoleak. The patient was operated on owing to the risk of rupture. Pelvic ischemia was a concern during decision-making. The patient underwent removal of the endograft and replacement of a bifurcated aortoiliac and femoral graft with good outcome. Midline laparotomy and a supraceliac clamping approach enable the removal of endografts with suprarenal fixation and revascularization of internal iliac arteries. Open repair offers a definitive solution for complicated endoleaks when endovascular options could be risky and ineffective.

First Successful Organ Procurement From a Pediatric Patient With a Nonpulsatile Ventricular Assist Device.

Left ventricular assist devices have become an important therapeutic option as a mechanical circulatory support system in the treatment of end-stage heart failure. Organ transplants from brain dead donors on mechanical circulatory support are rare. In the literature, many successful solid-organ transplants have been reported using these donors. However, to our knowledge, this is the first report of successful solid-organ transplant from a child donor with a nonpulsatile ventricular assist device.

First Harvest of Pediatric and Congenital Heart Surgery Multicenter Database in Turkey: Novel Application of Real-Time Online Reporting.

OBJECTIVES: A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. METHODS: The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. RESULTS: Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. CONCLUSIONS: CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

Association between caregivers' coping and children's psychiatric symptoms in the heart transplantation process: A pilot study.

Our aim was to investigate whether there is an association between caregivers' coping and children's psychiatric symptoms and quality of life in adolescent heart transplant (HTx) recipients and HTx candidates with left ventricular assist device (LVAD). Fourteen patients were recruited for this pilot study (HTx (n = 8), LVAD (n = 6)). Schedule for Affective Disorders and Schizophrenia for School Aged Children, Present and Lifetime Version (K-SADS) was administered to detect the psychiatric diagnosis of patients. Children's Depression Inventory (CDI), State-Trait Anxiety Inventory, and Pediatric Quality of Life Inventory (PedsQL) were completed by adolescents; Brief Coping Styles Inventory by their caregivers. Six of the participants had an internalizing disorder. Optimistic coping strategy score was significantly higher in the caregivers of adolescents without an internalizing disorder than caregivers of those with an internalizing disorder (U = 2.500, P = .005). Utilizing Spearman's correlation, caregivers' optimistic approach (rho = -0.736, P = .004), and self-confident approach (rho = -0.634, P = .020) had significant negative correlations with children's CDI scores. Moreover, caregivers' optimistic approach score had a significant positive correlation with children's PedsQL score (rho = 0.563, P = .045). According to our preliminary results, it seems that caregivers' optimistic and self-confident coping strategies may be associated with fewer internalizing symptoms and a better quality of life in adolescents in the HTx process. A future multicentered longitudinal study will be planned to assess the effect of caregivers' coping strategies on the psychological adjustment of these children.

Assessment of Tricuspid Valve Detachment Efficiency for Ventricular Septal Defect Closure: A Retrospective Comparative Study.

BACKGROUND: The aim of this study was to investigate the efficiency of tricuspid valve detachment (TVD) during the surgical treatment of perimembranous ventricular septal defects (VSDs) and to compare the early and mid-term results to patients without TVD in terms of tricuspid insufficiency. METHODS: A total of 170 patients who had undergone surgical closure of perimembranous VSDs between November 2012 and January 2019 were included in this study, of whom 50 had an additional TVD procedure during the surgery. All patients were examined by transthoracic echocardiography before and after the operation with regular intervals, and the tricuspid valve function was then evaluated. RESULTS: There was no significant difference between subgroups with an unchanging degree of TVR, however, the result was also similar among those who had a decreased degree of TVR at any level (p = 0.271, p = 0.451). At the end of the study, all patients were in New York Heart Association class I. CONCLUSIONS: We suggest that, in appropriate patients, VSD closure can be safely performed with an additional TVD application through an incision of the septal leaflet of the tricuspid valve without impairing the valve function or reducing the growth potential of the valve at midterm follow-up.

Long-term assessment of left ventricular ejection fraction and mitral regurgitation following takeuchi repair

Abstract Objective: This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA). Methods: Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 years) who were operated for ALCAPA between 2007 and 2018 were included in this study. Data were evaluated retrospectively based on our medical records. Results: Hospital mortality rate was 7.1% (n=1). Thirteen surviving patients were kept in follow-up mean 4.3±3.05 years. When compared to preoperative measurements, both left ventricular ejection fraction (LVEF), (P=0.007) and mitral regurgitation (MR) (P=0.001) significantly improved before discharge. Moreover, LVEF values were improved in the late follow-up, considering early postoperative outcomes, and this alteration was significant (P=0.014). Nevertheless, alteration in the degree of MR among patients did not differ in the long-term follow-up (P=0.180). There was no late-term mortality or need for reoperation among patients. Conclusion: Although some centers prefer to direct implantation in ALCAPA, Takeuchi procedure can be accepted as a reliable method that provides satisfactory long-term results, considering that it aids to improve left ventricle ejection fraction and reduced mitral valve regurgitation.

Long-Term Assessment of Left Ventricular Ejection Fraction and Mitral Regurgitation Following Takeuchi Repair.

OBJECTIVE: This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA). METHODS: Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 years) who were operated for ALCAPA between 2007 and 2018 were included in this study. Data were evaluated retrospectively based on our medical records. RESULTS: Hospital mortality rate was 7.1% (n=1). Thirteen surviving patients were kept in follow-up mean 4.3±3.05 years. When compared to preoperative measurements, both left ventricular ejection fraction (LVEF), (P=0.007) and mitral regurgitation (MR) (P=0.001) significantly improved before discharge. Moreover, LVEF values were improved in the late follow-up, considering early postoperative outcomes, and this alteration was significant (P=0.014). Nevertheless, alteration in the degree of MR among patients did not differ in the long-term follow-up (P=0.180). There was no late-term mortality or need for reoperation among patients. CONCLUSION: Although some centers prefer to direct implantation in ALCAPA, Takeuchi procedure can be accepted as a reliable method that provides satisfactory long-term results, considering that it aids to improve left ventricle ejection fraction and reduced mitral valve regurgitation.

Comparison Between Nomograms Used to Define Pediatric Aortic Arch Hypoplasia: Retrospective Evaluation Among Patients Less Than 1 Year Old with Coarctation of the Aorta.

Although various modalities are currently in use to define pediatric aortic arch hypoplasia (AAH), there is little uniformity among them. We aimed to determine the inter-rater strength of agreement of the nomograms in a survey of patients less than 1 year old, who had undergone coarctation of the aorta (CoA) repair with or without AAH. This retrospectively designed study comprised of 105 patients with CoA, who had been evaluated between 2008 and 2018 by means of a computed tomography angiogram. Through re-estimation of the aortic arch segmental diameters, the z scores were calculated using three nomograms (Cantinotti, Pettersen, Lopez). Along with a t test and Pearson's correlation coefficient, a linear regression analysis, Bland-Altman plots, and Cohen's kappa k value were used to evaluate inter-rater strength of agreement. The mean age and weight of the cohort was 73.3 ± 81.2 days and 4.2 ± 1.6 kg, respectively. Sixty-four (61%) patients were neonates. The z scores of the nomograms for each aortic arch segment were significantly different. Although there was a significantly positive correlation between the nomograms with their related aortic arch diameter, the differences in z scores revealed considerable deviations in the scatter plot diagrams. The mean difference of z scores was significantly different from the testing value of zero, which was also presented in Bland-Altman plots. None of the comparisons reached a kappa k value of > 0.9. The current nomograms do not reveal an acceptable level of agreement for the definition of the AAH. The question is which modality to rely on when deciding on the surgical approach and technique of CoA repair to address the hypoplastic aortic arch segment. Decisions about the surgical approach and the technique of repair warrant a reliable definition of AAH. It is high time that a consensus is reached in this regard.

Requirement for repetitive surgical approaches at supravalvular aortic stenosis.

Supravalvular aortic stenosis, which is a rare congenital cardiac anomaly, is associated with several lesions and has a progressive nature. Herein, we report a five-year-old girl with bicuspid aorta who underwent initial Doty operation at the age of nine months. A combined redo Doty operation and an aortic valve commissurotomy were performed two years later. Due to the rapidly progressing aortic regurgitation and both valvular and supravalvular gradient, a repeated surgery was required at the age of five years and an aortic homograft was successfully inserted with an annulus enlargement and the patient was discharged uneventfully. In conclusion, although Doty repair yields satisfactory results in most patients, certain cases with identified risk factors may require reoperations due to the progressive nature of the disease. Therefore, these patients should be kept under a close follow-up lifelong.

Blalock-Taussig Shunt Size: Should it be Based on Body Weight or Target Branch Pulmonary Artery Size?

The study aimed to revisit the in-hospital predictors of shunt thrombosis (ST) in the foreground of the pulmonary artery size in patients who received modified Blalock-Taussig shunt (mBTS) as the first-stage palliation. Data from 80 patients who received mBTS as their initial palliative procedure between February 2012 and January 2017 was retrospectively collected. The median age and weight of the patients at the time of their mBTS procedure was 4 days (IQR 2-22 days) and 3.2 kg (IQR 2.8-3.7 kg), respectively. Of the 80 patients in the study, 11 (13.8%) developed ST. The diameter and corresponding z scores of the pulmonary arteries were significantly lower in patients with ST. The median shunt size/shunted pulmonary artery size (S/PA) ratio was considerably higher in patients with ST. In logistic regression analysis, pulmonary artery hypoplasia (PAH) [odds ratio (OR) = 13.7 (0.06-0.21), p < 0.001], S/PA ratio ≥ 0.9 [OR = 8.1 (0.03-0.53), p = 0.03], prematurity [OR = 9.5 (0.05-0.33), p = 0.003], and shunt size/weight (S/W) ratio ≥ 1.3 [OR = 6.4 (0.04-0.67), p = 0.012] were found to have a significant impact on ST. The best combination of sensitivity and specificity of the S/W (0.73 and 0.75) and the S/PA ratio (0.73 and 0.80) were achieved at the cut-off value of 1.3 and 0.9, respectively. The Youden index of S/PA was 0.52. While the area under the curve (AUC) of the S/W ratio was 0.686 ± 0.12 (p = 0.049), the AUC of the S/PA ratio was 0.791 ± 0.08 (p = 0.002). In conclusion, instead of weight, considering the size of the target pulmonary artery and thereby, the S/PA ratio would be more instructive in determining shunt size. There were a high number of patients in our study who showed PAH having received a shunt size based on their body weight. By contrast, our results showed that the S/PA ratio of ≥ 0.9 would be a good predictor of in-hospital ST.

Correction to: Blalock-Taussig Shunt Size: Should it be Based on Body Weight or Target Branch Pulmonary Artery Size?

The original version of the article unfortunately contained an alignment error in Table 1. The correct version of Table 1 is given below.

Our experience on Bentall procedure in an adult patient with Baraitser-Winter syndrome.

Baraitser-Winter syndrome is a very rare genetic disorder caused by cytoplasmic actin-encoding genes defects. Although most patients have similar phenotype, concomitant cardiac anomalies widely vary. In addition to well-described congenital heart diseases, aortic aneurysms occur due to underlying actin gene mutation in these patients in the further years. Herein, we present a 26-year-old male case who underwent Bentall procedure with the diagnosis of new-onset aortic valve regurgitation and an ascending aorta aneurysm.

Surgical management of late-term pulmonary venous baffle stenosis after Senning operation.

Although pulmonary venous baffle stenosis is not a common late complication after the Senning operation, surgical treatment is vital, when interventional methods fail to correct the pathology. Our patient was admitted to hospital with recurrent epistaxis and exertional dyspnea 6.5 years after the Senning procedure. The gradient in the pulmonary venous baffle was measured as 34 to 35 mmHg via transthoracic echocardiography. During surgical treatment, the calcified and retracted polytetrafluoroethylene flap was completely excised and porcine pericardial xenograft was used to cover the pulmonary venous baffle. At the end of the procedure, no significant gradient was detected via transesophageal echocardiography.

Nonthermal, Nontumescent Endovenous Treatment of Varicose Veins.

BACKGROUND: Endothermal treatment of the great saphenous vein has become the first line of treatment for superficial venous reflux. A new technique for venous insufficiency is non-thermal ablation with vein sealing system which comprises the endovenous delivery of cyanoacrylate tissue adhesive to the vein causing fibrosis. METHODS: This is a single-center prospective study of treatment of great saphenous vein incompetence in 62 patients with vein sealing system (Biolas VariClose® FG Group, Turkey). All cases were implemented under local anesthesia. Tumescent anesthesia was not required. Patients were not given any nonsteroidal anti-inflammatory drug postoperatively; advised to wear elastic bandages for 1 day; and compression stockings were not offered. RESULTS: Treatment success was defined as complete occlusion of treated vein or recanalized segment shorter than 5 cm. Subtotal recanalization was defined as great saphenous vein flow containing 5-10 cm segment of treated vein. A recanalized great saphenous vein or treatment failure was defined as an open part of the treated vein segment more than 10 cm in length. At 1 week and 1 month control, duplex scans showed total occlusion for all patients (100%), total occlusion for 58 patients (93.5%), and subtotal occlusion for 4 patients (6.5%) at third month. At the end of 6 months, total occlusion 56 patients (90.3%) and subtotal occlusion for 2 patients (3.2%). For 4 (6.5%) patients, no occlusion was observed, and the diameter was >11 mm. Embolization of great saphenous vein with cyanoacrylate has been performed since the beginning of this decade. Combined chemical and physical mechanism of action results in permanent vein closure. In a recently published study, a 24-month occlusion rate of 92% was demonstrated. The most commonly reported complications of cyanoacrylate use for the treatment of varicose vein disease, so far, include ecchymosis and phlebitis. Almeida et al. reported that phlebitis is the most frequent side effect at a rate of 16%. In our study, phlebitis rate was not as high as reported. It may be caused due to shorter time of follow-up in the hospital. CONCLUSION: Endovenous ablation of incompetent great saphenous vein with cyanoacrylate-based glue is feasible. Operation time is short, and tumescent anesthesia is unnecessary as postprocedure compression stockings. Lack of significant side effects and an yearly success rate of 100% are benefits of the system.

The retrieval of atrial septal defect closure device embolized into aortic arch.

Percutaneous atrial septal defect (ASD) closure has become an increasingly simplified procedure over the past decade. The device embolization is seen rarely but it can be fatal. Although percutaneous retrieval is feasible, surgical removal might be preferred when the endothelialization status of the device is unknown. We report a comlication of such closure in a 43-year-old woman: embolization of the ASD occluder device into aortic arch 12 months after implantation. We removed the device surgically and closed the ASD.

Primary intravascular synovial sarcoma: case report.

Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 × 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.