Multimodality hemodynamic evaluation for optimizing management of anomalous origin of the right pulmonary artery from the aorta in an adolescent.

The hemodynamics of anomalous origin of the pulmonary artery (PA) from the aorta is challenging. Different sources of blood supply to the lungs lead to a unique state of differential flow, pressure, and pulmonary vascular resistance in each lung. The decision for surgical reimplantation of the anomalous PA during infancy is easy. The assessment of operability beyond infancy, however, is perplexing. In this report, we describe stepwise multimodal hemodynamic evaluation and successful surgical management in a 15-year-old boy with an isolated anomalous origin of the right PA from the aorta. We also report 5-year hemodynamic data confirming sustained benefit over the long term, thus providing much-needed clinical validation of often cited Poiseuille's and Ohm's laws.

Tetralogy of Fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery.

A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit.

Where are we after 50 years of the Fontan operation?

First introduced in 1971, the Fontan procedure is the final common destination for all patients with a functional single ventricle. The procedure itself has evolved tremendously over the last five decades. This review traces this journey and presents the importance, outcomes and future outlook of the procedure in the current era.

Systolic excursion of the leaflets of the truncal valve: An unusual mechanism for pulmonary stenosis in common arterial trunk.

BACKGROUND: Pulmonary stenosis in patients with common arterial trunk protects the pulmonary vasculature. In our recently published prospective study of common arterial trunk, some patients with sinusal origin of the pulmonary arterial segment had pulmonary stenosis induced by systolic excursion of a truncal valvar leaflet. We aimed to determine the detailed morphologic characteristics of this unusual finding. METHODS AND RESULTS: All 70 patients underwent echocardiography and computed tomographic angiography as per predefined study protocol. In selected cases, we also performed cardiac catheterization. Among 27 patients with aortic dominance, we found sinusal origin of the pulmonary arterial segment. In 5 of these patients, pulmonary stenosis was induced by systolic excursion of a truncal valvar leaflet. In all these patients, the truncal valve was trisinusate, albeit with asymmetric sinuses. The pulmonary arterial segment arose from the largest left sinus with its relatively large leaflet obstructing the pulmonary orifice during systole. In the remaining 22 patients, without pulmonary stenosis but with sinusal origin of pulmonary arterial segment, the truncal valve was quadrisinusate in 7, bisinusate in 2, and trisinusate in 13. None of the patients with quadrisinusate and bisinusate truncal valves had pulmonary stenosis. Among the 13 patients with trisinusate valves, the sinuses of the truncal valve were symmetrical in 6, while in 7, the pulmonary orifice originated from a smaller asymmetric sinus. CONCLUSION: Pulmonary stenosis, produced by a relatively large leaflet of an asymmetric truncal sinus, may prevent early development of pulmonary vascular disease. Timely recognition of this unusual mechanism of pulmonary stenosis is important for optimal management.

Extracardiac Fontan With Direct Inferior Vena Cava to Main Pulmonary Artery Connection Without Cardiopulmonary Bypass.

We report the case of a six-year-old patient who underwent an extracardiac Fontan operation including bilateral bidirectional superior cavopulmonary anastomosis and direct inferior vena cava to main pulmonary artery connection that was performed without cardiopulmonary bypass.

Double Outlet Right Ventricle With Right-Sided Aorta From the Left-Sided Morphologically Right Ventricle in the Setting of Discordant Atrioventricular Connections.

We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children's Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.

Clarifying the anatomy of common arterial trunk: a clinical study of 70 patients.

AIMS: Anatomic variations in hearts with common arterial trunk are well-known, although there is no large study of living patients. Detailed knowledge of the origins of the pulmonary and coronary arteries is vital for surgical management. We sought to clarify the variations using computed tomography. METHODS AND RESULTS: We prospectively studied 70 consecutive patients using echocardiography and computed tomography. In 63 (90%) patients, there was aortic dominance, while 7 (10%) had dominance of the pulmonary component. In 27 (43%) patients with aortic dominance, part of the pulmonary segment arose from a truncal valvar sinus. A long confluent pulmonary channel was more common in patients with sinusal origin compared to those with non-sinusal origin of the pulmonary segment (19 vs. 0; P = 0.0005). Close proximity between the orifices of the coronary arteries and the pulmonary component was also more frequent with sinusal origin (21 vs. 6; P < 0.001) with 5 (19%) patients having pulmonary flow obstructed by a truncal valvar leaflet. CONCLUSION: Sinusal origin of the pulmonary component is common with aortic dominance, frequently in association with a long confluent pulmonary segment, which may be in close proximity to the origin of a coronary artery. One-fifth of patients with sinusal origin of pulmonary component have a truncal valvar leaflet obstructing the pulmonary orifice. These morpho-anatomic findings have important implications for management.

Adipose tissue derived mesenchymal stem cells are better respondents to TGFß1 for in vitro generation of cardiomyocyte-like cells.

Mesenchymal stem cells (MSCs) are multipotent cells which hold immense potential in translational research as a novel treatment modality. In recent years, MSCs isolated from various tissues have been used in several clinical trials for the treatment of cardiac injury caused by permanent myocardial loss. However, a better MSCs source and an optimum inducer for in vitro cardiac differentiation are still far reaching and unexplored. The aim of the study was to compare the ability and efficiency of differentiation of MSCs isolated from bone marrow (BM-MSCs) and adipose tissue (ADSC) into cardiomyocyte-like cells to aid translational research. To fulfill this aim, freshly isolated BM-MSCs and ADSCs were differentiated into cardiomyocytes using 5-Azacytidine (6 µM) and TGF-ß1 (25 ng/ml). These two differentiation protocols were compared on the basis of morphological, transcriptional, translational and functionality analysis. Both tissue specific MSCs, ADSCs and BM-MSCs, have similar surface marker profile and population doubling time. In both the treatment regimes, BM-MSCs and ADSCs showed morphological changes like flattening of cells and myotube formation in concurrence with structure and multinucleation, with early sign of differentiation in ADSCs. Further, the expression of cardiac specific markers including myosin light chain-2v (Mlc-2v), cardiac troponin I (cTnI), and sarco/endoplasmic reticulum Ca2+-ATPase (SerCa2) were higher in AD-TGFß1 group, both at transcriptional and translational level. During functionality analysis by KCl stimulation, increased intracellular calcium fluorescence was observed in AD- TGFß1 group as compared to others. Thus, ADSCs proved to be a better choice for stem cell therapy in cardiovascular diseases when induced with TGF-ß1.

An alternative technique for completion of the total cavopulmonary connection.

BACKGROUND: Total Cavopulmonary connection (Fontan) is the final palliation for patients with a functionally univentricular heart. This is commonly accomplished after a prior bidirectional Glenn on cardiopulmonary bypass (CPB) with separate cannulation of the aorta, superior vena cava (SVC), and inferior vena cava. We describe an alternative technique of Fontan completion that eliminates the need for cannulation and dissection of the SVC, and pulmonary artery dissection. METHODS: Between January and October 2018, 17 patients underwent completion Fontan using an alternate technique at our institute. All operations were conducted on CPB at normothermia without cannulating the SVC RESULTS: Mean CPB time was 60 ± 16.8 minutes (range, 39-102 minutes). There were no early deaths. Mean postoperative Fontan pressures were 15.6 ± 1.2 mm Hg with no gradient between the SVC and IVC pressures. Mean duration of hospital stay was 15.6 ± 3.6 days (range, 10-22 days). No patient developed phrenic nerve paresis or palsy. CONCLUSIONS: Completion without cannulating the SVC is simple, reproducible, and easy to teach. It avoids the disadvantages associated with routine techniques.

Early right ventricular function following trans-right atrial versus trans-right atrial, trans-right ventricular repair of Tetralogy of Fallot: Results of a prospective randomized study.

OBJECTIVE: We compared the pre- and post-operative right ventricular (RV) function by tricuspid annular plane systolic excursion (TAPSE) between trans-right atrial (t-RA) versus t-RA/RV (RA/RV) approach for the repair of Tetralogy of Fallot (TOF). PATIENTS AND METHODS: Fifty consecutive patients, 1-15 years of age, undergoing intracardiac repair of TOF between September 2015 and June 2016 were randomized into two groups based on the approach for repair as follows: t-RA or t-RA/RV approach. TAPSE was used for the assessment of pre- and post-operative RV function. RESULTS: Age, body surface area, preoperative saturation, cardiopulmonary bypass and aortic cross-clamp times, inotropic score, postoperative intensive care unit, and hospital stay were similar in both the groups. However, t-RA/RV group had significant mediastinal drainage (169 ± 163 ml vs. 90.6 ± 58.7 ml, P < 0.05) and pleural effusions (8 vs. 2 patients, P < 0.05), but had better relief of RV outflow tract gradients. The mean follow-up was 23 ± 6.7 (median 26, range 21-29) months. There were no differences in arrhythmias in either group up to the 1st month and at last follow-up. Preoperative TAPSE for t-RA and t-RA/RV was similar (1.49 ± 0.29 vs. 1.66 ± 0.34, P > 0.05) and so was the post-operative TAPSE at discharge (1.52 ± 0.30 vs. 1.43 ± 0.32, P > 0.05), at 1 month (1.6 ± 0.27 vs. 1.43 ± 0.032, P > 0.05) and at last follow-up (1.79 ± 0.15, median 1.8 vs. 1.72 ± 0.17, median 1.7 P > 0.05). CONCLUSION: Both t-RA and t-RA/RV approaches provide safe palliation for patients with TOF. A limited right ventriculotomy neither leads to deleterious effects on early RV function nor does it increase the incidence of arrhythmias at early follow-up. Larger studies with longer follow-up are needed to further address these issues.

Patch materials for right ventricular outflow reconstruction: past, present, and future.

In this review, we discuss various patch materials used for reconstruction of the right ventricular outflow tract. Their relative merits and demerits are discussed. Traditional patches and their results are detailed along with a brief description of newer developments in the field.

Comparison of del Nido and histidine-tryptophan-ketoglutarate cardioplegia solutions in pediatric patients undergoing open heart surgery: A prospective randomized clinical trial.

OBJECTIVES: We conducted a prospective randomized controlled trial to compare del Nido (DN) and histidine-tryptophan-ketoglutarate (HTK) cardioplegia solution in pediatric patients undergoing intracardiac tetralogy of Fallot repair. METHODS: One hundred consecutive patients 12 years of age or younger, undergoing intracardiac repair of tetralogy of Fallot were randomized into DN (n = 50) and HTK (n = 50) groups. Cardioplegia strategy consisted of a single dose of DN (20 mL/kg) or HTK (6 mL/kg/min for 6 minutes). Primary outcome was cardiac index (CI). Secondary outcomes were ventricular arrhythmias post cross-clamp release, time to peripheral rewarming, duration of mechanical ventilation, inotropic score, intensive care unit and hospital stay, and serum levels of troponin-I, interleukin-6, and tumor necrosis factor-α. Ultrastructural changes in the myocardium were assessed. RESULTS: CI was significantly higher in the DN group compared with the HTK group at 6 (P = .005) and 24 hours (P < .001) after surgery. It was on an average 0.44 L/min/m2 higher in the DN group at any time point (P = .004). Time for complete cessation of electrical activity was longer in the HTK group (P = .01) and more patients in the HTK group had ventricular arrhythmias post cross-clamp release (P = .03). Duration of mechanical ventilation (P = .006), intensive care unit stay (P = .05), and hospital stay (P < .001) were lower in the DN group. Patients in the DN group had lower troponin I levels 24 hours after cardiopulmonary bypass (P < .001). Electron microscopic studies showed more myocardial edema (P = .02) and myofibrillar disarray (P = .04) in the HTK group along with lower glycogen stores (P = .04). DN cardioplegia was more cost-effective than HTK cardioplegia (P < .001). CONCLUSIONS: DN cardioplegia was associated with better preservation of CI, less duration of mechanical ventilation, shorter intensive care unit and hospital stays, lower inotropic scores, and less release of troponin-I. Electron microscopy showed less myocardial edema and better preservation of the myofibrillar architecture and glycogen stores in the DN group.

Oral thyroxin supplementation in infants undergoing cardiac surgery: A double-blind placebo-controlled randomized clinical trial.

BACKGROUND: Decreases in serum total thyroxin and total triiodothyronine occurs after cardiopulmonary bypass, and is reflected as poor immediate outcome. We studied effects of oral thyroxin supplementation in infants who underwent open-heart surgery. METHODS: In this prospective study, 100 patients were randomized into 2 groups: 50 in the thyroxin group (TH) and 50 in the placebo group (PL). Patients in the TH group received oral thyroxin (5 µg/kg) 12 hours before surgery and once daily for the remainder of their intensive care unit (ICU) stay. Data on intraoperative and postoperative variables were recorded. Cardiac index (CI) was measured. Perioperative serum thyroid hormone levels and serum interleukin-6 and tumor necrosis factor-α were measured. Secondary analysis was performed by dividing patients into simple and complex subcategories. RESULTS: Results of the primary analysis indicated a higher CI in the TH compared with the PL. In the complex category, the mean duration of mechanical ventilation was 3.85 ± 0.93 and 4.66 ± 1.55 days in the TH and PL, respectively (P = .001). Mean ICU stay was 6.79 ± 2.26 and 8.33 ± 3.09 days (P = .03), and mean hospital stay was 15.70 ± 4.77 and 18.90 ± 4.48 days (P = .01) in the TH and PL, respectively. There were no significant differences between the TH and the PL in the simple category. CI was higher in the TH at all time points (P = .004). The average therapeutic intervention scoring system scores for the first 2 days were higher in the PL in the complex category. CONCLUSIONS: Oral thyroxin supplementation improves the CI and reduces the inotropic requirement. In addition, it reduces the duration of mechanical ventilation, ICU and hospital stay, and therapeutic intervention scoring system in infants after surgery for complex congenital heart defects.

Multiple saccular aortic aneurysms following the arterial switch operation.

We report a 3-month-old male presenting with multiple aortic aneurysms arising de novo 2 months following the arterial switch operation. Successful repair of the aneurysms was performed under total circulatory arrest and at seven years follow-up, the patient has no recurrence.

Exercise performance after univentricular palliation.

BACKGROUND: The optimal timing, need for primary/staged procedure in patients undergoing univentricular palliation, is debatable. AIMS: We performed this study to assess the exercise performance of patients undergoing various forms of univentricular palliation. SETTING AND DESIGN: This was a retrospective, prospective comparative study conducted at a multispecialty tertiary referral center. PATIENTS AND METHODS: Between January 2012 and June 2015, 117 patients undergoing either bidirectional Glenn (BDG) (n = 43) or Fontan (total cavopulmonary connection [TCPC]) (n = 74) underwent exercise testing. STATISTICAL ANALYSIS: Comparisons between subgroups for continuous data were made with Student's t-test if normally distributed and Wilcoxon rank-sum test otherwise. Tests between subgroups for qualitative data were made with Pearson's Chi-square test. RESULTS: Patients who underwent BDG with open antegrade pulmonary blood flow (APBF) had higher saturations (oxygen saturation [SpO2]) compared to those without it (87.5 ± 5.0% vs. 81.1 ± 4.8%; P = 0.0001). However, we found no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC (n = 42) patients demonstrated better exercise capacity (15.0 ± 7.7 vs. 11.2 ± 6.2 min; P = 0.02) and increased SpO2 on exercise (87.0 ± 8.0% vs. 83.4 ± 7.6%; P ≤ 0.05) compared to lateral tunnel TCPC (n = 32). Fenestrated TCPC (n = 30) patients had higher exercise capacity reflected by higher metabolic equivalents (METs) consumption (6.4 ± 2.3 vs. 5.2 ± 2.0 METs, P = 0.02), fewer pleural effusions (7.0 ± 3.2 vs. 9.2 ± 6.2 days, P ≤ 0.05), and lower hospital stay (9.5 ± 4.0 vs. 12.7 ± 7.7 days, P = 0.04) compared to nonfenestrated TCPC (n = 44) patients. CONCLUSIONS: We observed no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC patients had better exercise capacity but longer postoperative hospital stay and pleural effusions than patients with lateral tunnel Fontan. Fenestrated TCPC patients seemed to fare better than nonfenestrated ones. Patients undergoing TCPC had better exercise capacity than patients undergoing BDG alone.

A prospective study of risk factors associated with persistent pleural effusion after total cavopulmonary connection with special reference to serum cortisol level.

OBJECTIVES: The Fontan operation is usually followed by significant pleural effusion. We aimed to study the factors associated with persistent pleural effusion with special reference to serum cortisol levels. PATIENTS AND METHODS: Thirty-eight patients undergoing the Fontan operation between September 2015 and November 2016 were prospectively studied. Parameters studied included age, weight, symptoms, atrio- ventricular valve regurgitation/stenosis/atresia, ventricular function, pulmonary artery pressures, oxygen saturation, aorto-pulmonary, and veno-venous collaterals, type of Fontan, duration of cardiopulmonary bypass, need for inotropes, duration of mechanical ventilation, conduit size, presence or absence of fenestration, and serum cortisol levels. The latter were measured before and after the Fontan operation and the co-relation between pleural effusion and change in serum cortisol levels was studied. RESULTS: Mean age at operation was 13.1 ± 5.6 years (median 13 years). Mean duration and amount of pleural drainage was 15.76 ± 13.2 days (median 11.5 days) and 9.15 ± 4.6 mL/kg/day (median 9 mL/kg/day) respectively. Statistically significant risk factors for prolonged pleural effusion were higher pulmonary artery (PA) pressures (r = 0.328, p = 0.003, odds ratio 1.30), higher inotropic score (r = 0.4, p = 0.01), lower rise in serum cortisol (p = 0.03),elevated superior caval venous pressure (CVP) at 6 h (r = 0.44, p = 0.005) and 12 h (r = 0.4, p = 0.01) and higher duration of mechanical ventilation (r = 0.45, p = 0.005). CONCLUSIONS: PA pressures > 15 mmHg, higher inotropic score, higher CVP and lower rise in serum cortisol levels following the Fontan operation were associated with persistent pleural effusion.

One and half ventricle repair: rationale, indications, and results.

Surgical strategies in patients with functionally or anatomically borderline right ventricles include a high-risk biventricular repair, a Fontan procedure, or a one and half ventricle repair (also referred to as the partial biventricular repair). One and half ventricle repair (1.5VR) circumvents the high early mortality of a biventricular repair and also the late morbidity of the Fontan. The two most common indications for a 1.5VR are a small pulmonary ventricle and a dilated poorly functioning pulmonary ventricle. Extension of 1.5VR to patients undergoing anatomical repair for congenitally corrected transposition of great arteries, straddling tricuspid valves, and severe Ebstein's anomaly has facilitated biventricular repair with decreased mortality. We reviewed the relevant literature on this subject in detail and describe its rationale, indications and its early and late results.

An unusual combination of discreet subaortic membrane, aortopulmonary window, severe aortic insufficiency and rheumatic mitral regurgitation.

We report a 15-year-old female patient with an unusual combination of discreet subaortic membrane, aortopulmonary window, severe aortic insufficiency and rheumatic mitral regurgitation.

Factors determining early outcomes after the bidirectional superior cavopulmonary anastomosis.

OBJECTIVE: The bidirectional Glenn (BDG) procedure is a step in multistage palliation of univentricular heart (UVH). We aimed to report the factors determining the outcomes following BDG. METHODS: Two hundred fifteen consecutive patients, 5.29 ± 5 years (range 1 month to 38 years, median 3 years) of age, weighing 13 ± 8.8 kg (range 2.6 to 51 kg, median 10 kg) with variable forms of UVH underwent BDG from 2003 to 2013. Their clinical records were reviewed retrospectively. RESULTS: The most common anatomic diagnoses were tricuspid atresia (n = 87, 40.5%) and double outlet right ventricle (n = 78, 36%). Dextrocardia was present in 21 (9.86%) patients. Median left pulmonary (PA) and right PA diameters were 6 and 7 mm, respectively. One hundred sixty-two (77%) patients received unilateral BDG, and 45 had bilateral BDG. The antegrade pulmonary blood flow was closed in 199 and was left open in 16 patients. Concomitant procedures were reconstruction of pulmonary arteries for non-confluent PA (n = 28), atrial septectomy (n = 15), atrioventricular valve repair (n = 12) and repair of partial anomalous pulmonary venous connection (n = 1). A total of 37% of patients (n = 80) had a mean post-operative saturation of 90 ± 3.2%. There were four (1.86%) early deaths. Mean Glenn pressure was 14.7 ± 3.5 mm Hg, and mean inotropic score and Vasoactive inotropic score (VIS) were 1.64 ± 0.96 and 2.77 ± 2.63, respectively. Mean intensive care unit stay was 24.1 ± 26.4 (range 10-240) h, and mean duration of hospital stay was 7.15 ± 3.2 days. Mean saturation at the time of discharge was 92.4 ± 2.2% and on follow-up was 82 ± 2.16%. Follow-up cardiac catheterization data was available in 123 (60.3%). Sixty-nine (33.8%) patients underwent completion Fontan, and 135 patients were in follow-up or waiting for Fontan completion. CONCLUSION: BDG procedure can be performed safely with acceptable mortality. Age at presentation, pulmonary artery size and VIS were not related to mortality. Younger patients had similar outcomes but a longer hospital stay. Patients with preserved antegrade pulmonary blood flow had higher saturations. Those undergoing BDG without cardiopulmonary bypass had lower inotropic scores.

Immediate and early post-operative sequelae of off-pump total cavopulmonary connection.

BACKGROUND: Extracardiac Fontan (ECF) is currently the final operation of choice for patients with a univentricular heart. Performing this procedure without cardiopulmonary bypass (CPB) carries potential benefits. In this study, we report the early results of ECF without CPB. PATIENTS AND METHODS: Between 2012 and 2015, 72 consecutive patients underwent Fontan without CPB. Their medical records were examined in detail. RESULTS: Mean age was 11.8 ± 5.2 (range 5 to 23, median 10) years. Intraoperative mean superior vena cava clamp time was 15.19 ± 3.8 min, and the inferior vena cava clamp time was 16.93 ± 3.31 min. There were three early deaths. No patient required conversion from off-CPB to CPB. Mean inotropic score was 4.73 ± 5.9 (range 0 to 25, median 2.5). Mean time to extubation was 9.5 ± 5.82 (range 3 to 29, median 8) hours. Pleural drainage in intensive care unit (ICU) was 551.57 ± 452.77 (median 470) ml, and mean ICU stay was 2.27 ± 3.09 (median 1.5) days. Mean daily pleural drainage after discharge from the ICU was 163.7 ± 88.01 (median 140) ml, and mean time to removal of pleural tubes was 15.76 ± 8.4 (median 14) days. Total hospital stay was 17.03 ± 8.62 (median 15) days. At an early follow-up of 2-40 (median 25) months, all survivors (n = 69) had a patent Fontan circuit with normal ventricular function on echocardiography. There were no late deaths or thromboembolic complications. CONCLUSIONS: Off-pump ECF is a low-risk procedure that avoids the harmful effects of CPB. Post-operative course of these patients is predictable with substantial savings in costs.