The Fate of the Left Ventricular Outflow Tract Following Interrupted Aortic Arch Repair.

Objectives: To examine the probability of left ventricular outflow tract (LVOT) reintervention following interrupted aortic arch (IAA) repair in neonates with LVOT obstruction (LVOTO) risk. Methods: This retrospective multicenter study included 150 neonates who underwent IAA repair (2003-2017); 100 of 150 (67%) had isolated IAA repair (with ventricular septal defect closure) and 50 of 150 (33%) had concomitant LVOT intervention: conal muscle resection (n = 16), Ross-Konno (n = 7), and Yasui operation (n = 27: single-stage n = 8, staged n = 19). Demographic and morphologic characteristics were reviewed. Factors associated with LVOT reoperation were explored using multivariable analysis. Results: Concomitant LVOT intervention was more likely in neonates with type B IAA, bicuspid aortic valve, aberrant right subclavian artery, smaller aortic valve annulus, and ascending aorta dimensions. On follow-up, five-year freedom from LVOT reoperation was highest following Ross-Konno (100%), 77% following Yasui (mainly for neo-aortic regurgitation), 77% following isolated IAA repair (mainly for LVOTO), and 47% following IAA repair with concomitant conal resection, P = .033. While all patients had low peak LVOT gradient at time of discharge, those who had conal resection developed higher gradients on follow-up (P = .007). Ross-Konno and Yasui procedures were associated with higher right ventricular outflow tract (RVOT) reoperation. In the cohort following isolated IAA repair, aortic sinus Z score was associated with LVOT reoperation. Conclusions: Both Yasui and Ross-Konno operations effectively mitigate late LVOTO risk. The highest risk of reintervention for LVOTO was associated with conal muscle resection while the lowest risk is associated with Ross-Konno. The RVOT reoperation risk in patients who had Ross-Konno or Yasui does not seem to affect survival.

Double outlet left atrium: successful management of a rare entity.

Double-outlet left atrium is an extremely rare congenital ventriculo-atrial mal-alignment anomaly, wherein, the left atrium drains into both ventricles, through either a common atrioventricular valve or two separate atrioventricular valves. The only egress from the right atrium is through an inter-atrial communication. We present a 16-month-old male, diagnosed to have double outlet left atrium and describe its surgical management.

Experience with surgical correction of double outlet both ventricles.

Double outlet both ventricles is a rare abnormal ventriculo-arterial malformation in which both great arterial trunks are committed to both the ventricles, albeit now being recognized with increasing frequency. Patients with the lesion present with a spectrum of clinical manifestations. The size and location of the interventricular communication dictate the feasibility of biventricular repair. Literature on the malformation, however, is sparse. We report our experience with five patients, all of whom underwent successful surgical biventricular repair.

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

Aortic valve repair in the pediatric population: emerging role of aortic valve neocuspidization (AVNeo procedure).

Management of aortic valve diseases in children is challenging owing to the quality and quantity of the native tissue for repair, limitations in the currently available biological materials to supplement the repair and to achieve a long-lasting durable repair in an annulus where there is still growth potential. The aortic valve neocuspidization (AVNeo) procedure has emerged as a versatile alternative strategy in the armamentarium of pediatric aortic valve reconstructions that are currently available. In this review article, the focus of the discussion will be on the various aortic valve repair procedures in the pediatric population, with a special emphasis on the emerging role of AVNeo procedure in pediatrics and its outcomes.

Comparison between Del Nido and conventional blood cardioplegia in pediatric open-heart surgery.

BACKGROUND: Del Nido cardioplegia (DNc) was designed for superior myocardial protection during cardiopulmonary bypass (CPB). We conducted a retrospective review to explore if DNc was associated with increase in systemic ventricle dysfunction (sVD) following pediatric CPB. METHODS AND RESULTS: This single-center, retrospective study included 1534 patients undergoing CPB between 2013 and 2016, 997 prior to center-wide conversion to DNc and 537 following. The primary outcome was new postoperative ≥moderate sVD by echocardiogram. Secondary outcomes included sVD of any severity and right ventricular dysfunction. Data was evaluated by interrupted time-series analysis. Groups had similar cardiac diagnoses and surgical complexity. Del Nido cardioplegia was associated with longer median (IQR) CPB [117 (84-158) vs 108 (81-154), p = 0.04], and aortic cross-clamp [83 (55-119) vs 76 (53-106), p = 0.03], and fewer cardioplegia doses [2 (1-2) vs 3 (2-4), p < 0.0001]. Mortality was similar in both groups. Frequency of sVD was unchanged following DNc, including predetermine subgroups (neonates, infants, and prolonged cross-clamp). Logistic regression showed a significant rise in right ventricular dysfunction (OR 5.886 [95% CI: 0.588, 11.185], p = 0.03) but similar slope. CONCLUSIONS: Use of DNc was not associated with increased in reported sVD, and provided similar myocardical protection to the systemic ventricle compared to conventional cardioplegia but may possibly impact right ventricular function. Studies evaluating quantitative systolic and diastolic function are needed.

Circumflex aortic arch: presentation across various age groups: a case series.

Circumflex aortic arch (CAA) is a rare congenital anomaly where the aortic arch crosses the midline, posterior to the esophagus and trachea, and descends on the contralateral side. If patent ductus arteriosus (PDA) is present, this forms a true vascular ring. CAA can compress the trachea and esophagus leading to a myriad of symptoms which can present at any age. We describe our experience with three patients of the CAA, presenting across different age groups.

Intrapulmonary artery septation for pulmonary artery growth in functionally univentricular hearts.

The pulmonary artery anatomy is one of the important determinants of Fontan completion and its outcomes in patients with functionally univentricular hearts. Despite several modifications to Fontan operation, unbalanced pulmonary arteries pose a significant challenge in this subset of patients. Intrapulmonary artery septation is a technique, developed to rehabilitate the unilateral hypoplastic pulmonary artery following various initial palliative procedures in functionally univentricular hearts. Literature is sparse on this technique. This article reviews the basic concepts and literature on intrapulmonary artery septation. We also describe our experience with modified intrapulmonary artery septation.

Double aortic arch with atretic proximal left arch.

Aortic arch anomalies are rare and represent about 1% of all congenital cardiac lesions. Double aortic arch with atretic proximal left arch is one of the rare causes of respiratory symptoms in neonates, often missed by preoperative imaging studies. Preoperative identification and differentiating this entity from other arch anomalies is imperative to plan appropriate surgical management.

Branch pulmonary artery stenosis after arterial switch operation: The effect of preoperative anatomic factors on reintervention.

BACKGROUND: We hypothesized that preoperative patient characteristics and branch pulmonary artery (PA) size might influence the rate of postoperative branch PA reintervention in patients with transposition of the great arteries who undergo the arterial switch operation (ASO). METHODS: The retrospective single-center study included 262 consecutive (2008-2017) newborns who underwent the ASO. Demographic characteristics, echocardiography, and clinical outcomes were reviewed. Competing risk analysis modeled incidence of branch PA reintervention and cause-specific hazard regression for predictors analyses. RESULTS: Median age and weight were 7 (range, 5-11) days and 3.4 (range, 3.1-3.8) kg, respectively. Various types of early branch PA reinterventions (concomitant revision or reintervention during the intensive care unit stay) were required in 28 (10.7%) patients. These patients had prolonged ventilation (P < .001), intensive care unit duration (P < .001), worse right ventricular function (P = .043), and high in-hospital mortality (P = .010). Branch PA dimensions significantly decreased immediately after ASO compared with baseline measurements. The median follow-up duration was 20.8 (range, 0.9-44.7) months. Branch PA reintervention was common among survivors without early reinterventions (9.4%), and even more frequent among those with early reinterventions (25%). Subsequent reintervention (all catheter-based) was necessary for more than one-third of patients after initial branch PA reintervention. The multivariable analysis showed preoperative dimension of the left PA (hazard ratio, 0.527 [95% CI, 0.337-0.823]; P = .005), and right PA (hazard ratio, 0.503 [95% CI, 0.318-0.796]; P = .003) were independently associated with late branch PA reinterventions. CONCLUSIONS: Branch PA reintervention was common and often required surgical or catheter-based reinterventions after ASO. PA branch diameters became significantly smaller after ASO. Smaller preoperative branch PA predicted late branch PA reintervention, indicating a smaller margin of geometrical tolerance to this effect.

Circumflex aorta: An uncharted territory.

BACKGROUND: Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons. METHODS: A comprehensive search of peer reviewed journals was completed based on the key words, "Circumflex aorta," "Circumflex retro-esophageal aorta" and "circumflex arch" using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies. RESULTS: Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported. CONCLUSION: Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.

Aortic valve neocuspidization for aortic regurgitation associated with ventricular septal defect.

OBJECTIVES: Different methods of aortic valve repair have been described in the literature for aortic regurgitation (AR) associated with doubly committed subarterial ventricular septal defects. Our goal was to present our experience with aortic valve reconstruction of a single leaflet using the aortic valve neocuspidization technique in this subset of patients. METHODS: It is a retrospective review of 7 patients with doubly committed subarterial ventricular septal defects with significant (>moderate) AR who underwent the single-leaflet neocuspidization technique of aortic valve reconstruction from January 2016 to January 2019. Data were collected from medical records. All patients had thorough 2-dimensional echocardiographic assessment preoperatively and during the follow-up period. Primary end points were freedom from postoperative AR and freedom from reoperation and all-cause mortality within the follow-up period with secondary end points of freedom from thromboembolism and infective endocarditis. RESULTS: Out of 7 patients, 6 were male and 1 was female. There were no perioperative deaths. The mean follow-up period was 2.6 ± 0.8 years. No deaths occurred during the follow-up period. At the latest follow-up examination, only 2 patients showed mild AR and were asymptomatic. There was no documented event of infective endocarditis or thromboembolism during the follow-up period. CONCLUSIONS: The aortic leaflet neocuspidization procedure for the aortic valve is a relatively new concept. Availability of a template makes it an easily reproducible valve repair in paediatric patients with a single-leaflet abnormality. This technique preserves the remaining 2 normal leaflets, thus promoting the growth potential while maintaining near normal aortic root complex dynamics.

Aortic uncrossing with concomitant arch augmentation in a rare case of left circumflex aorta with arch hypoplasia.

Retro-esophageal circumflex aortic arch is a rare form of vascular ring. Majority of cases occur in isolation without any intracardiac lesions. Only a few cases of left circumflex aorta have been reported in the literature. We present a case of left circumflex aorta with associated arch hypoplasia and coarctation, ventricular septal defect, who underwent single-stage repair.

Iatrogenic closure of coronary sinus: an unforseen complication of surgical atrial septal defect closure.

Cardiac complication following the surgical closure of a secundum atrial septal defect (ASD) is extremely uncommon in the current era. We report a case of 39-year-old male presented with hemoptysis following an ASD closure 21 years prior and diagnosed to have iatrogenic closure of the coronary sinus at the time of surgical ASD closure. To our best knowledge, this is the first case of such complication reported in the literature.

Berry syndrome-a rare congenital cardiac anomaly.

Berry syndrome is a rare congenital cardiac anomaly, characterized by distal aortopulmonary window, hypoplasia or interruption of the aortic arch, intact ventricular septum, and aortic origin of the right pulmonary artery and patent ductus arteriosus. Anatomic depiction of each component is important for the diagnosis. Single-stage surgical repair is challenging but feasible with good survival outcomes. The available literature on this anomaly is limited. Hence, this paper aims at reviewing the literature on Berry syndrome.

A rare cause of left ventricular outflow tract mass in infancy: Cardiac papillary fibroelastoma.

Primary cardiac tumors are one of the rare causes of intracardiac masses in pediatric population. Cardiac papillary fibroelastomas are benign cardiac tumors with predilection for valvular endocardium, and the presentation is dictated by the cardiac structure involved and the tumor characteristics. We report an infant with cardiac papillary fibroelastoma manifesting as an asymptomatic left ventricular outflow tract mass. This case is being presented to emphasize the rare occurrence of cardiac papillary fibroelastoma in pediatric population and to be cognizant of this entity when considering differential diagnosis of intracardiac masses in this subgroup.