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Objectives: One of the most common cyanotic congenital heart diseases seen in India is the tetralogy of Fallot (TOF). The presence of chronic hypoxia leads to increased susceptibility to ischemia and infections. The postoperative morbidity and mortality can be predicted earlier, by incorporating various biochemical markers in pre-operative workup, which can minimize post-operative mechanical ventilation and intensive care unit (ICU) stay. We aimed to study 11 different cardiac biomarkers and calculate the All India Institute of Medical Sciences (AIIMS) score as a prognostic marker in TOF patients. Material and Methods: After obtaining Institute Ethics Committee approval from the Hospital Ethics Committee with Indian Council of Medical Research (ICMR) Trial No: 5/4/1-1/08-NCD-II and written informed consent, a prospective and observational study was conducted on 150 patients with TOF undergoing elective intra cardiac repair (ICR) divided into two groups. Anesthetic and surgical management was standardized for all patients as per institutional protocol. The data were analyzed in STATA software. The sample size was calculated on the basis of the area under the curve for various biomarkers shown in the previous literature reviews. Results: There was a positive correlation between the Endothelin levels 48 h after bypass and post-operative outcome measures such as the duration of inotropes, duration of ventilation, and duration of ICU stay. Pre-cardiopulmonary bypass serum tumor necrosis factor-alpha (TNF-α) showed a significant correlation with mortality in group I patients (P = 0.009) and group II patients (P < 0.05). Intragroup comparison in survivors showed significant changes with time in lactate trends. The mean initial post-operative lactate was significantly lower for survivors than for non-survivors. In addition, the serial mean lactate decreased progressively in all surviving patients compared with non-survivors diagnostic receiver operating characteristic curve for the pressure of oxygen. Conclusion: The four biomarkers, namely, Endothelin, TNF-α, BNP, and base excess, were found to be highly sensitive and specific. Using these biomarkers, a score of 2.73 (the AIIMS TOF score) is considered morbid in patients post-ICR in the ICU. The chances of mortality are high, with a sensitivity of 96.9% and specificity of 89.2%. [ABSTRACT FROM AUTHOR]

Background and Aim: To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re‐interventions, for the rare instances of transposition physiology with concordant ventriculo‐arterial connections. Methods: We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo‐arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Results: Among reported cases, just over two‐thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four‐fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one‐fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one‐fifth of patients requiring pacemakers for surgically‐induced heart block. The majority of survivors were in good functional state. Conclusions: The flow patterns produced by discordant atrioventricular and concordant ventriculo‐arterial connections remain an important, albeit rare, indication for atrial redirection or hemi‐Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long‐term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The most severe combinations of cardiac malformations exist in individuals having jumbled-up thoracic and abdominal organs. These patients make up 2 distinct syndromes. As yet, the consensus is lacking on how best to describe the subsets. The subsets are frequently grouped together in terms of 'heterotaxy'. The surgical approaches to the subsets, however, are markedly different. We reviewed our experiences with regard to the anatomy as observed in the autopsy room, by the analysis of computed tomographic studies, and in the operating room, to assess whether the lesions might be segregated on the basis of isomerism of the atrial appendages. METHODS AND RESULTS A review of our findings from the examination of specimens from several archives, along with investigation of a large cohort of patients being prepared for surgical treatment, showed that individuals can uniformly be segregated into subgroups on the basis of isomeric arrangement of the atrial appendages. In all instances, this was made possible by using the criterion of the extent of the pectinate muscles within the appendages as judged relative to the atrial vestibules. Segregation on this basis, which correlated excellently with the bronchial arrangement, sets the scene for an appropriate description of the remainder of the heart, providing the cardiac surgeon with all the inferences required for appropriate surgical intervention. CONCLUSIONS When assessing individuals having the features of so-called 'heterotaxy', it is possible to segregate the groups into subsets of individuals having either isomeric right or left atrial appendages. This approach provides the framework for the assessment of appropriate surgical management. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The phenotypic features and morphogenesis of the 'sinus venosus defect' remain controversial. The phenotypic features are anomalous systemic connections of 1 or more pulmonary veins that retain their left atrial connection, usually associated with a biatrial connection of the superior caval vein. Cases with these features, however, have not always been described as sinus venosus defects. METHODS We reviewed the findings documented in the literature from 11 patients with a biatrial connection of the superior caval vein, most reported following an autopsy examination. We compared these findings with the anatomical details of 50 patients undergoing surgical correction in our centre, paying particular attention to the override of the superior caval vein. RESULTS In only two-thirds of those undergoing surgery did the superior caval vein override the rims of the oval fossa, with the degree of override >50% in only 2 individuals. It is only these latter 2 cases that are directly comparable to the reported cases of biatrial connection of the superior caval vein. CONCLUSIONS Our comparisons provide new insights into the developmental background and phenotypic features of the superior sinus venosus defect. The defects exist because of the anomalous systemic connection of the pulmonary veins that retain their left atrial connections but not always in association with a biatrial connection of the superior caval vein. In extreme cases, nonetheless, they can underscore the connection of the caval vein to the morphologically left atrium, frequently described previously as a 'biatrial connection'. The sinus venosus defect is better considered a venovenous malformation than a septal defect. [ABSTRACT FROM AUTHOR]

Background and Aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages. Methods: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts. Results: Half of patients with right isomerism, and two‐thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three‐quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two‐fifths of those with left isomerism. Univentricular atrioventricular connections are expected in up to three‐quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four‐fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic‐to‐pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one‐quarter had undergone Fontan completion, with reported mortalities of 21%. Conclusion: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival. [ABSTRACT FROM AUTHOR]

Introduction  Extracorporeal membrane oxygenation (ECMO) is increasingly being used in refractory cardiac and pulmonary dysfunction as a rescue modality. The common indications for establishing venoarterial ECMO (VA-ECMO) support in children postcardiac surgery are failure to wean from cardiopulmonary bypass (CPB), postcardiotomy cardiogenic shock (PCCS), refractory pulmonary arterial hypertension, and as a bridge to recovery or transplant. The survival rate of children on VA-ECMO support is 45%. The most frequently encountered complications during VA-ECMO are bleeding, thrombosis, acute kidney injury, and infections. Among those, infections acquired during VA-ECMO lead to high morbidity and mortality. Hence, this study aimed to determine infection rates, causal microorganisms, and mortality risk factors in children developing an infection during VA-ECMO therapy. Methods  This retrospective observational study was conducted on 106 children under 14 years of age who underwent elective or emergent cardiac surgery (between 2016 and 2020) and required VA-ECMO support. Medical records were reviewed to collect the targeted variables and analyzed. Results  Out of 106 children, 49 (46.23%) acquired infections representing a prevalence of 46.23% and an infection rate of 186.4 episodes per 1,000 ECMO days. Prevalence and acquired infection rate/1,000 ECMO days were higher in the nonsurvivor group than in the survivor group (26.42 vs.19.81%) and (215.07 vs. 157.49), respectively. The bloodstream infection (BSI) and catheter-associated urinary tract infection (CAUTI) episodes were 53.04 and 68.19 per 1,000 ECMO days, and the ventilator-associated pneumonia (VAP) rate was 44.50 per 1,000 ventilator days. The mean preoperative admission duration, aortic cross-clamping duration, CPB duration (minutes), and vasoactive-inotropic score were higher in the nonsurviving children (p  < 0.001). Similarly, prolonged mean ECMO duration was also found in the nonsurvivor group compared with the survivor group (p  = 0.03). Conclusion  In our study, the prevalence of acquired infection during VA-ECMO was 46.23%. The incidence of BSI, CAUTI, and VAP per 1,000 ECMO days was higher in the nonsurvivor group than in survivors. Acinetobacter baumannii was the most common cultured gram-negative organism in VAP and BSI, with 67.65% Acinetobacter spp. resistant to carbapenems. CAUTI was predominately due to Candida species during VA-ECMO. [ABSTRACT FROM AUTHOR]

We sought to ascertain the short- and long-term results of total pericardiectomy for chronic constrictive pericarditis using a modified left anterolateral thoracotomy without cardiopulmonary bypass on postoperative low cardiac output, normalization of intracardiac pressures, survival, and reoperations. Between January 2005 and December 2019 a series of 127 consecutive patients (91 male patients) between ages 4 and 72 years (median, 25 years; interquartile range, 18-38) underwent radical total pericardiectomy using a modified left anterolateral thoracotomy without cardiopulmonary bypass. Operative and late mortalities were 3.1% and 1.6%, respectively. Thirty-one patients (24.4%) had postoperative low cardiac output, and none required reoperations. At a median follow-up of 99 months (interquartile range, 56-141) the actuarial survival was 97.6% ± 0.01% months (95% confidence interval, 92.8-99.2). At their last follow-up 113 (93.4%) and 8 (6.6%) survivors were in New York Heart Association class I and II, respectively. Total pericardiectomy is associated with lower perioperative and late mortality and decreased low cardiac output syndrome and confers significant long-term advantage of superior hemodynamics. [ABSTRACT FROM AUTHOR]

We evaluated aortic tissue specimens from patients undergoing tetralogy of Fallot repair, to determine whether histologic abnormalities affect postsurgical aortic remodeling and other patient-related variables. Using light microscopy, we studied full-thickness aortic wall tissue operatively excised from 118 consecutive patients undergoing intracardiac repair of tetralogy of Fallot. We performed multiple linear regression analysis to identify independent predictors of change in aortic root dimensions, which we measured with echocardiography after repair and every 3 months thereafter. Thirty histologically normal specimens were used as controls. Elastic fiber fragmentation was found in 74.6% of the abnormal specimens, mucoid extracellular matrix accumulation in 49.2%, smooth muscle cell nuclei loss in 39%, smooth muscle cell disorganization in 28.8%, and medial fibrosis in 52.5%. At a mean follow-up time of 83.55 ± 42.08 months, mean aortic sinotubular diameter decreased from 28.79 ± 9.15 to 27.16 ± 8.52 mm/m2 (r =–0.43; P <0.001). Aortic sinotubular diameter decreased by 0.6 mm/m2 among females (β =0.6, SE=0.31; P =0.05) and by 0.88 mm/m2 in patients who had elastic fiber fragmentation or loss (β =0.88, SE=0.38; P =0.02). In bivariate and multiple linear regression analysis, duration of follow-up emerged as an independent predictor of aortic remodeling. The aortic histopathologic changes in our patients had an independent negative impact on the degree of aortic remodeling after surgery. We observed the most improved aortic sinotubular diameter in patients who had either histologically normal aortas or aortas with elastic fragmentation. [ABSTRACT FROM AUTHOR]

Background: Serum lactate and base deficit have been shown to be a predictor of morbidity and mortality in critically ill patients. Poor preoperative oxygenation appears to be one of the significant factors that affects early mortality in tetralogy of Fallot (TOF). There is little published literature evaluating the utility of serum lactate, base excess (BE), and oxygen partial pressure (PO 2 ) as simple, widely available, prognostic markers in patients undergoing surgical repair of TOF.Materials and Methods: This prospective, observational study was conducted in 150 TOF patients, undergoing elective intracardiac repair. PO 2 , BE, and lactate levels at three different time intervals were recorded. Arterial blood samples were collected after induction (T1), after cardiopulmonary bypass (T2), and 48 h (T3) after surgery in the Intensive Care Unit (ICU). To observe the changes in PO 2 , BE, and lactate levels over a period of time, repeated measures analysis was performed with Bonferroni method. The receiver operating characteristics (ROC) analysis was used to find area under curve (AUC) and cutoff values of various biomarkers for predicting mortality in ICU.Results: The patients who could not survive showed significant elevated lactate levels at baseline (T1) and postoperatively (T2) as compared to patients who survived after surgery (P < 0.001). However, in nonsurvivors, the BE value decreased significantly in the postoperative period in comparison to survivors (-2.8 ± 4.27 vs. 5.04 ± 2.06) (P < 0.001). In nonsurvivors, there was a significant fall of PO 2 to a mean value of 59.86 ± 15.09 in ICU (T3), whereas those who survived had a PO 2 of 125.86 ± 95.09 (P < 0.001). The ROC curve analysis showed that lactate levels (T3) have highest mortality predictive value (AUC: 96.9%) as compared to BE (AUC: 94.5%) and PO 2 (AUC: 81.1%).Conclusion: Serum lactate and BE may be used as prognostic markers to predict mortality in patients undergoing TOF repair. The routine analysis of these simple, fast, widely available, and cost-effective biomarkers should be encouraged to predict prognosis of TOF patients. [ABSTRACT FROM AUTHOR]

Stenotrophomonas maltophilia is an emerging gram-negative pathogen that was previously labeled as a colonizer. Nowadays, with multiple antibiotic usage along with certain host factors, infections caused by this organism are getting attention. We hereby report two cases of ventilator-associated pneumonia in postoperative infants by Stenotrophomonas maltophilia in a cardiac intensive care unit (ICU). [ABSTRACT FROM AUTHOR]

Objectives: To investigate the release pattern of different cardiac metabolites and biomarkers directly from the coronary sinus (CS) and to establish the diagnostic discrimination limits of each marker protein and metabolites to evaluate perioperative myocardial injury in patients undergoing cardiac surgery under cardiopulmonary bypass (CPB).Patients and Methods: Sixty-eight patients undergoing first mitral and/or aortic valve replacements with/without coronary artery bypass grafting and Bentall procedure under CPB and blood cardioplegic arrest were studied. All cardiac metabolites and biomarkers were measured in serial CS-derived blood samples at pre-CPB, immediate post aortic declamping, 10 minutes post-CPB and 12 hrs post-CPB.Results: Receiver operating characteristic curve analysis of cardiac biomarkers indicated lactate-pyruvate ratio as the superior diagnostic discriminator of myocardial injury with an optimal "cut-off" value >10.8 immediately after aortic declamping (AUC, 0.92; 95% CI: 0.85-0.98). Lactate was the second best diagnostic discriminator of myocardial injury with an optimal "cut-off" value >2mmol/l at immediately after aortic declamping (AUC, 0.89; 95% CI: 0.80-0.96). Cardiac troponin-I was the third best diagnostic discriminator of myocardial injury with an optimal "cut-off" value >2.1ng/ml at immediately after aortic declamping (AUC, 0.88; 95% CI: 0.80-0.95). Creatine kinase-MB was the fourth best diagnostic discriminator of myocardial injury with an optimal "cut-off" value >58 log units/ml prior to decanulation (AUC, 0.85; 95% CI: 0.78-0.94).Conclusions: Measurable cardiac damage exists in all patients undergoing cardiac surgery under cardioplegic arrest. The degree of myocardial injury is more in patients with poor ventricular function and those requiring longer aortic clamp time. CS-derived lactate-pyruvate ratio, lactate, cTn-I served as superior diagnostic discriminators of peri-operative myocardial damage. [ABSTRACT FROM AUTHOR]

Introduction: Hypoalbuminemia is a well-recognized predictor of general surgical risk and frequently occurs in patients with cyanotic congenital heart disease (CCHD). Moreover, cardiopulmonary bypass (CPB)-induced an inflammatory response, and the overall surgical stress can effect albumin concentration greatly. The objective of his study was to track CPB-induced changes in albumin concentration in patients with CCHD and to determine the effect of hypoalbuminemia on postoperative outcomes.Materials and Methods: Prospective observational study conducted in 150 patients, Group 1 ≤18 years (n = 75) and Group 2 >18 years (n = 75) of age. Albumin levels were measured preoperatively (T1), after termination of CPB (T2) and 48 h post-CPB (T3). Primary parameters (mortality, duration of postoperative ventilation, duration of inotropes and duration of Intensive Care Unit [ICU] stay) and secondary parameters (urine output, oliguria, arrhythmias, and hemodynamic parameters) were recorded.Results: The albumin levels in Group 1 at T1, T2, and T3 were 3.8 ± 0.48, 3.2 ± 0.45 and 2.6 ± 0.71 mg/dL; and in Group 2 were 3.7 ± 0.50, 3.2 ± 0.49 and 2.7 ± 0.62 mg/dL respectively. All patients showed a significant decrease in albumin concentration 48 h after surgery (P < 0.01). Analysis between the groups, however, showed no statistical difference. Eleven patients expired during the study period, and nonsurvivors showed significantly lower serum albumin concentration 48 h after surgery 2.3 ± 0.62 mg/dL versus 3.7 ± 0.56 mg/dL in the survivors (P < 0.05). Receiver operating characteristic curve showed that a baseline albumin cut-off value of 3.3 g/dL predicts mortality with a positive predictive value 47.6% and a negative predictive value of 99.2% (P < 0.05). A strong correlation was seen between albumin levels at 48 h with duration of CPB (r2 = 0.6321), ICU stay (r2 = 0.7447) and incidence of oliguria (r2 = 0.8803).Conclusions: The study demonstrated similar fall in albumin concentration in cyanotic patients (both adult and pediatric) in response to CPB. Low preoperative serum albumin concentrations (<3.3 g/dL) can be used to identify and prognosticate subset of cyanotics predisposed to additional surgical risk. [ABSTRACT FROM AUTHOR]

Background: The identification of biomarkers for predicting morbidity and mortality, particularly in pediatric population undergoing cardiac surgery will contribute toward improving the patient outcome. There is an increasing body of literature establishing the clinical utility of hyperlactatemia and lactate clearance as prognostic indicator in adult cardiac surgical patients. However, the relationship between lactate clearance and mortality risk in the pediatric population remains to be established.Objective: To assess the role of lactate clearance in determining the outcome in children undergoing corrective surgery for tetralogy of Fallot (TOF).Methods and Study Design: A prospective, observational study.Setting: A tertiary care center.Study Population: Two hundred children undergoing elective surgery for TOF.Study Method: Blood lactate levels were obtained as baseline before operation (T0), postoperatively at admission to the cardiac intensive care unit after surgery (T1), and then at every 6 h for the first 24 h of Intensive Care Unit (ICU) stay (T6, T12, T18, and T24, respectively). The lactate clearance in the study is defined by the equation ([lactate initial - lactate delayed]/lactate initial) ×100%. Lactate clearance was determined at T1-T6, T1-T12, T1-T18, and T1-T24 time interval, respectively. The primary outcome measured was mortality. Secondary outcomes measured were the duration of mechanical ventilation, duration of inotropic requirement, and duration of ICU stay.Results: Eleven out of the two hundred patients enrolled in the study died. Nonsurvivors had higher postoperative lactate concentration (P < 0.05) and low-blood lactate clearance rate during 24 h (P < 0.05) in comparison to the survivors. Lactate clearance was significantly higher in survivors than in nonsurvivors for the T1-T6 period (19.55 ± 14.28 vs. 5.24 ± 27.79%, P = 0.009) and remained significantly higher for each studied interval in first 24 h. Multivariate logistic regression analysis of statistically significant univariate variables showed early lactate clearance to have a significant relationship with mortality. Patients with a lactate clearance >10%, relative to patients with a lactate clearance <10%, in the early postoperative period, had improved outcome and lower mortality.Conclusion: Lactate clearance in the early postoperative period (6 h) is associated with decreased mortality rate. Patients with higher lactate clearance (>10%) after 6 h have improved outcome compared with those with lower lactate clearance. [ABSTRACT FROM AUTHOR]

Severe persistent hypertension is seen infrequently in newborns and infants, but we came across two infants who developed severe paradoxical hypertension after successful coarctation repair. Treatment of systemic hypertension following repair of coarctation of the aorta is always challenging particularly in infants. Dexmedetomidine was used successfully as an adjunct to the established anti-hypertensive drugs in the immediate postoperative period in our cases to treat postoperative paradoxical hypertension. [ABSTRACT FROM AUTHOR]

A 50-year-old woman with rheumatic heart disease, mitral stenosis, and critical isolated left main ostial stenosis was successfully treated by mitral valve replacement, tricuspid annuloplasty, and surgery of left main osteoplasty and is reported for its rarity. Notable clinical findings included an intermittently irregular pulse, blood pressure of 100/70 mmHg, cardiomegaly, a diastolic precordial thrill, a mid-diastolic murmur without presystolic accentuation that was loudest at the mitral area. Chest radiograph revealed cardiomegaly with a cardiothoracic ratio of 0.7 due to enlarged right atrium, right ventricle with a straightened left heart border and evidence of pulmonary hypertension. The investigation shows that surgical reconstruction of the left main coronary artery is safe and effective for the treatment. [ABSTRACT FROM AUTHOR]

We report a rare complication of massive aneurysm of the proximal ligated end of the main pulmonary artery which occurred in the setting of a patient with a functionally univentricular heart and increased pulmonary blood flow undergoing superior cavopulmonary connection. Awareness of this possibility may guide others to electively transect the pulmonary artery in such a clinical setting. [ABSTRACT FROM AUTHOR]

An eleven-year-old boy expectorated a foreign body in cough that was identified as the prosthetic graft used for a Blalock-Taussig shunt. The shunt procedure was done 10 years earlier, and a definitive repair for tetralogy of Fallot was done a year later. He had no other symptoms, and a computed tomography (CT) angiogram did not reveal any other significant anomaly. The reason for this extremely rare event is unclear. [ABSTRACT FROM AUTHOR]

Background: This study was designed to evaluate patients aged less than 40 years implanted with tissue heart valves with respect to survival, thromboembolism, structural degeneration and quality of life.Methods: Between January, 2000 and December, 2016, 132 patients (51 males) with rheumatic heart disease underwent mitral valve replacement using Carpentier-Edwards, perimount, pericardial bioprostheses. The patients' ages ranged between 12 and 39 years (mean±SD 30.12±5.51 years).Results: The hospital and late mortality were 1.5% and 1.5% respectively. The total cumulative follow-up period was 1330.98 patient-years with a mean of 124.78±50.3 months (range, 1-204 months). The actuarial survival and actuarial event-free survival at 204 months was 96.9% (±0.01%) and 93.4%(±0.03%) respectively. There was one episode of thromboembolism (0.32 events per 100 patient years). Six (4.7%) patients underwent redo mitral valve replacement for severe bioprosthetic degeneration with stiffening and calcification using a Medtronic mechanical prosthesis (Medtronic Open Pivot, MN, USA).Conclusions: We conclude that Carpentier-Edwards perimount pericardial prosthesis provides satisfactory clinical performance in a young population with a low risk of degeneration and other valve-related events. [ABSTRACT FROM AUTHOR]

The phenotypic feature of tetralogy of Fallot is anterocephalad deviation of the muscular outlet septum, or its fibrous remnant, relative to the septoparietal trabeculation, coupled with hypertrophy of septoparietal trabeculations. Although this feature permits recognition of the entity, no two cases are identical. Once diagnosed, treatment is surgical. The results of surgical treatment have improved remarkably over recent decades. The results are now sufficiently excellent, including those in the developing world, that attention is now directed toward avoidance of morbidity, while still seeking, of course to minimize any fatalities due to surgical intervention. It is perhaps surprising that attention thus far has not been directed on the potential significance of phenotypic variation relative to either mortality or morbidity subsequent to surgical correction. The only study we have found specifically addressing this variability focused on the extent of aortic override, and associated malformations, but made no mention of variability in the right ventricular margins of the interventricular communication, nor the substrates for subpulmonary obstruction. In this review, therefore, we assessed the potential significance of known morphological variability to the outcomes of surgical intervention in over 1,000 individuals undergoing correction by the same surgeon in a center of excellence in a developing country. We sought to assess whether the variations were associated with an increased risk of postoperative death, or problems of rhythm. In our hands, double outlet ventriculoarterial connection was associated with increased risk of death, while the presence of a juxta-arterial defect with perimembranous extension was associated with rhythm problems., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. Methods: Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. Results: The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism ( P  < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism ( P  = .006). Conclusions: Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.

Objectives: The most severe combinations of cardiac malformations exist in individuals having jumbled-up thoracic and abdominal organs. These patients make up 2 distinct syndromes. As yet, the consensus is lacking on how best to describe the subsets. The subsets are frequently grouped together in terms of 'heterotaxy'. The surgical approaches to the subsets, however, are markedly different. We reviewed our experiences with regard to the anatomy as observed in the autopsy room, by the analysis of computed tomographic studies, and in the operating room, to assess whether the lesions might be segregated on the basis of isomerism of the atrial appendages., Methods and Results: A review of our findings from the examination of specimens from several archives, along with investigation of a large cohort of patients being prepared for surgical treatment, showed that individuals can uniformly be segregated into subgroups on the basis of isomeric arrangement of the atrial appendages. In all instances, this was made possible by using the criterion of the extent of the pectinate muscles within the appendages as judged relative to the atrial vestibules. Segregation on this basis, which correlated excellently with the bronchial arrangement, sets the scene for an appropriate description of the remainder of the heart, providing the cardiac surgeon with all the inferences required for appropriate surgical intervention., Conclusions: When assessing individuals having the features of so-called 'heterotaxy', it is possible to segregate the groups into subsets of individuals having either isomeric right or left atrial appendages. This approach provides the framework for the assessment of appropriate surgical management., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The phenotypic features and morphogenesis of the 'sinus venosus defect' remain controversial. The phenotypic features are anomalous systemic connections of 1 or more pulmonary veins that retain their left atrial connection, usually associated with a biatrial connection of the superior caval vein. Cases with these features, however, have not always been described as sinus venosus defects., Methods: We reviewed the findings documented in the literature from 11 patients with a biatrial connection of the superior caval vein, most reported following an autopsy examination. We compared these findings with the anatomical details of 50 patients undergoing surgical correction in our centre, paying particular attention to the override of the superior caval vein., Results: In only two-thirds of those undergoing surgery did the superior caval vein override the rims of the oval fossa, with the degree of override >50% in only 2 individuals. It is only these latter 2 cases that are directly comparable to the reported cases of biatrial connection of the superior caval vein., Conclusions: Our comparisons provide new insights into the developmental background and phenotypic features of the superior sinus venosus defect. The defects exist because of the anomalous systemic connection of the pulmonary veins that retain their left atrial connections but not always in association with a biatrial connection of the superior caval vein. In extreme cases, nonetheless, they can underscore the connection of the caval vein to the morphologically left atrium, frequently described previously as a 'biatrial connection'. The sinus venosus defect is better considered a venovenous malformation than a septal defect., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Competing Interests: Disclosures of Conflicts of Interest: N.N.P. No relevant relationships. V.P. No relevant relationships. P.J. No relevant relationships. U.K.C. No relevant relationships.

We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.

Background: Repair of superior sinus venosus defect with high partially anomalous pulmonary venous connection (PAPVC) using an intracardiac baffle may be complicated by systemic or pulmonary venous pathway obstruction and sinus nodal dysfunction (SND). Our surgical strategy for repair of all types of superior sinus venosus defect has evolved chiefly to avoid the abovementioned complications and preserving the growth potential of the superior cavoatrial junction., Methods: Between 2007 and 2019, fifty consecutive patients aged 2 to 60 (mean, 17.6±16.7) years underwent repair of superior sinus venosus defect using the double-barrel technique as described. The anomalous pulmonary veins drained into the superior cavoatrial junction in 17 patients and more than 2 cm above the cavoatrial junction in 33 patients., Results: There were no early or late deaths and no reoperations. At a mean follow-up of 103.9 (±50.2) months, all survived the operation, and actuarial freedom from SND was 97.9% (±standard error, 0.02%; 95% CI: 0.86-0.99). No patient had systemic or pulmonary venous pathway obstruction. A permanent pacemaker was required in one (2%) patient for sick sinus syndrome., Conclusions: The double-barrel method is an expedient, safe, and effective technique in superior sinus venosus defect. It provides dual drainage of superior vena cava preserving the superior cavoatrial junction without causing systemic or pulmonary venous pathway obstruction and can be utilized in all cases including those with high PAPVC. Preservation of the cavoatrial junction and use of autogenous atrial tissue for systemic venous pathway avoids SND and preserves growth potential.

The present perspective is a synthesis of published investigations in the setting of anomalous connection of the right superior caval vein to the morphologically left atrium or biatrial drainage of the right caval vein. We identified 57 suitable cases from 97 investigations, reviewing the clinical presentation, diagnostic modalities utilized, surgical techniques used, and their outcomes. Clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, radionuclide perfusion scan, magnetic resonance imaging, and angiocardiography provided the diagnostic information and were used to define the disease entities before surgery. We have also addressed several issues concerning the influence of the so-called heterotaxy: the establishment of the diagnosis, the variation in clinical presentation, and subsequent management. For the overall group of patients undergoing either surgical intervention or transcatheter treatment with an Amplatzer vascular plug, the operative mortality remains high at 9.5%. We submit that an increased appreciation of these disease entities will contribute to improved future surgical management.

We report two male patients aged 18 and 19 years, respectively, undergoing total pericardiectomy for chronic calcific constrictive pericarditis who developed systemic ventricular failure unresponsive to medical management following surgery. The failing circulation was successfully reestablished using intra-aortic balloon counterpulsation. Aortic counterpulsation facilitates recovery of ventricular function and appears to be a reasonable alternative in select instances of refractory cardiac failure following pericardiectomy.

Familial homozygous hypercholesterolemia is a rare disease with diverse clinical presentations ranging from premature ischemic heart disease to aortic root stenosis but rarely presents with anginal symptoms due to supravalvular and valvular aortic stenosis. We report a 19-year-old male patient with familial homozygous hypercholesterolemia with progressive supravalvular and valvular aortic stenosis that ultimately required aortic root enlargement and aortic valve replacement using a mechanical prosthesis, despite aggressive medical therapy. Surgical importance of this rare condition is highlighted.

The present perspective is a synthesis of published investigations in the setting of subvalvular aortic aneurysms. We identified 75 investigations and reviewed the clinical presentation, diagnostic modalities used, surgical techniques employed, and their outcomes. Clinical presentation, radiographic findings, transthoracic and transesophageal echocardiography, electrocardiogram-gated computerized tomography, and magnetic resonance imaging provided the diagnostic information and were used to define the disease entity before surgery. In this article, we have attempted to address several issues concerning establishment of diagnosis, varied clinical presentation, and their management. We submit that an increased appreciation of this disease entity will contribute to improved surgical management.

A 21-year-old female patient with repaired tetralogy of Fallot and aortic valvular reconstruction with aneurysmal aortic root and severe aortic regurgitation underwent aortic root replacement. Intrinsic aortopathy in tetralogy of Fallot and its surgical importance are highlighted.

Objectives: This study was designed to prospectively investigate the effects of pericardiectomy via median sternotomy on intra- and postoperative hemodynamics by a new semi-invasive device (Flotrac/VigileoTM monitor) using arterial pressure waveform analysis., Patients and Methods: Thirty consecutive patients aged 15 to 55 years (mean+SD, 31.73 + 13.53 years), who had undergone total pericardiectomy via median sternotomy underwent serial hemodynamic evaluation. FlotracTM Sensor - derived stroke volume, stroke volume variation, systemic vascular resistance index (SVRI), cardiac index and right atrial pressure were measured just before and after pericardiectomy, at 12 hours, 24 hours, 48 hours, 72 hours and at discharge postoperatively., Results: Majority of patients (73.33%) exhibited statistically significant reduction of right atrial pressure and SVRI along with improvement in cardiac index and oxygen delivery in the immediate and late postoperative period. However, the stroke volume and stroke volume variation did not increase proportionately on completion of surgery. Patients with low cardiac output syndrome exhibited persistently high central venous pressure with reduced cardiac index and echocardiographically abnormal diastolic filling characteristics., Conclusions: We conclude that there is early normalization of hemodynamics following pericardiectomy via median sternotomy and the adequacy of pericardiectomy can be accurately assessed by the new semi-invasive arterial pressure waveform analysis device. Stroke volume variation is a non-predictor of fluid requirement during and after pericardiectomy.

Objectives: To investigate the release pattern of different cardiac metabolites and biomarkers directly from the coronary sinus (CS) and to establish the diagnostic discrimination limits of each marker protein and metabolites to evaluate perioperative myocardial injury in patients undergoing cardiac surgery under cardiopulmonary bypass (CPB)., Patients and Methods: Sixty-eight patients undergoing first mitral and/or aortic valve replacements with/without coronary artery bypass grafting and Bentall procedure under CPB and blood cardioplegic arrest were studied. All cardiac metabolites and biomarkers were measured in serial CS-derived blood samples at pre-CPB, immediate post aortic declamping, 10 minutes post-CPB and 12 hrs post-CPB., Results: Receiver operating characteristic curve analysis of cardiac biomarkers indicated lactate-pyruvate ratio as the superior diagnostic discriminator of myocardial injury with an optimal "cut-off" value >10.8 immediately after aortic declamping (AUC, 0.92; 95% CI: 0.85-0.98). Lactate was the second best diagnostic discriminator of myocardial injury with an optimal "cut-off" value >2mmol/l at immediately after aortic declamping (AUC, 0.89; 95% CI: 0.80-0.96). Cardiac troponin-I was the third best diagnostic discriminator of myocardial injury with an optimal "cut-off" value >2.1ng/ml at immediately after aortic declamping (AUC, 0.88; 95% CI: 0.80-0.95). Creatine kinase-MB was the fourth best diagnostic discriminator of myocardial injury with an optimal "cut-off" value >58 log units/ml prior to decanulation (AUC, 0.85; 95% CI: 0.78-0.94)., Conclusions: Measurable cardiac damage exists in all patients undergoing cardiac surgery under cardioplegic arrest. The degree of myocardial injury is more in patients with poor ventricular function and those requiring longer aortic clamp time. CS-derived lactate-pyruvate ratio, lactate, cTn-I served as superior diagnostic discriminators of peri-operative myocardial damage.

Stem cell technology is rapidly gaining popularity as a way to improve the prognosis of patients with coronary artery disease and heart failure. In this review, we systematically analyze the basis, methods, and results of stem cell technology for coronary artery disease at the All India Institute of Medical Sciences, New Delhi, India.

Aneurysm formation of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines on optimal treatment. A 20-year-old woman with a huge saccular aneurysm of the main pulmonary artery, underwent repair with a pericardial patch and concomitant reconstruction of the pulmonary valve. The patient was doing well on follow-up at 6 months; echocardiography revealed a good repair with mild to moderate pulmonary regurgitation.