Assessing operability in late-presenting ventricular septal defects (VSD) with pulmonary arterial hypertension (PAH) is complex. Although a comprehensive assessment is often recommended, pulmonary vascular resistance index (PVRI) is the cornerstone of most decisions. PVRI-based operability assessment, nonetheless, is not infallible. Cases with PVRI much higher than operability cut-offs have done well, while some with lower PVRI have experienced late recurrence of PAH. We propose the Diastolic Pulmonary Shunt Index (DiPSI), defined as the diastolic pulmonary gradient (DPG = PADP - PAWP) divided by Qp/Qs, as an adjunct to aid clinical judgment. DiPSI, by offering a physiologically grounded, flow-adjusted index, may help clarify borderline cases, providing a more nuanced assessment of operability in late presenters with large VSD than PVRI alone.

Background:Extrinsic left main coronary artery (LMCA) compression is well described in patients with severe pulmonary dilatation secondary to atrial septal defect, idiopathic pulmonary artery hypertension, and eisenmengerized patent ductus arteriosus. An inferiorly displaced origin of LMCA closer to the left coronary sinus and a dilated pulmonary artery (PA) trunk twice as much as aortic trunk increases risk of extrinsic compression. Such patients are prone to left ventricular ischaemia, malignant ventricular arrhythmia, and sudden cardiac death. Case summary:A 20-year-old girl presented with gradually worsening exertional dyspnoea for the last 5 years was diagnosed to have tetralogy of Fallot with absent pulmonary valve (TOF-APV). After undergoing intra-cardiac repair with bioprosthetic pulmonary valve implantation, she developed a broad complex right bundle branch block with right precordial Q waves (qRBBB), new-onset left ventricular dysfunction, acute decompensated heart failure, and elevated serum cardiac enzymes. Coronary angiogram and intravascular ultrasound examination showed a critically narrowed slit-like LMCA ostium. Percutaneous stenting of LMCA saw resolution of qRBBB and improvement in left ventricle function, after which patient stabilized and could be weaned off ventilatory support. Discussion:Left main coronary artery compression after surgical pulmonary valve replacement in TOF-APV occurring few days after the operation may pose diagnostic and therapeutic challenges. Exact mechanism for post-operative compression is not known. It can be hypothesized that the ionotropic agents used in post-operative period, or change in the geometric relationships of pulmonary artery and LMCA due to pulmonary valve implantation, brought out the compression. A case for prophylactic PA size reduction can be made to avoid the possibility of LMCA compression.

Background Survival beyond one month of age is rare in children born with obstructed infracardiac total anomalous pulmonary venous connection (TAPVC). There are limited data available on surgical outcomes of the same subset. We conducted this retrospective study to identify risk factors associated with surgical outcomes in this patient population. Method We present a retrospective study of patients presenting at our institute with obstructed infracardiac TAPVC surviving beyond one month of age during the period from January 2015 to August 2019 (n = 14). Data reviewed included demographic parameters, preoperative laboratory results, echocardiogram images, and computed tomography if performed. Intraoperative as well as postoperative data including type of surgery, use of deep hypothermic arrest, mortality, and morbidity were noted. Follow-up clinical data were also collected from institutional record or telephonically. We have also collected echocardiographic data on follow up. Results The mean age of our study cohort was 2.14 ± 0.68 months. Out of 14 total patients 3 patients eventually died. Risk factors that were significantly associated with mortality were low weight (odds ratio [OR] = 7.8, P = .005), preoperative bilirubin level (OR = 7.4, P = .007), use of deep hypothermic circulatory arrest (OR = 4.6, P = .031), tricuspid regurgitation grade (OR = 3.8, P = .050), and pulmonary artery hypertension (OR = 10.5, P = .001). Mean follow up for the study cohort was 2189 ± 538 days. One patient has mild flow acceleration across the anastomosis with a gradient of 5/3 mm Hg without any symptoms. The remainder of the patients are alive with excellent clinical as well as echocardiographic outcome. Conclusion The presentation of infants with obstructed infracardiac TAPVC beyond one month of age is very rare. However, timely intervention can provide acceptable early and excellent late outcomes in this rare patient cohort.

A 13-year-old girl developed infective endocarditis (IE) on the native mitral valve after hospitalization for osteomyelitis. The clinical course was complicated by intramyocardial abscess and severe mitral regurgitation. Blood cultures were positive for Stenotrophomonas maltophilia, a rare cause of IE, which is known to be resistant to the first-line agents used for the treatment of IE, and has a propensity to cause a myocardial abscess. Hence, an increased awareness regarding endocarditis due to S. maltophilia is warranted.

OBJECTIVE:Ductal stenting (DS) has emerged as a critical intervention for neonates with duct-dependent pulmonary circulation (DDPC), offering a less invasive alternative to surgical shunts. METHODS:This retrospective study evaluates the procedural and mid-term outcomes of neonatal DS at a high-volume tertiary cardiac centre between January 2018 and August 2023. RESULTS:The study involved 124 symptomatic neonates. Primary outcomes included procedural success, defined as achieving post-procedural oxygen saturation (SpO2) ≥85 %, and survival to planned surgical repair. Secondary outcomes assessed included unplanned re-interventions, pulmonary artery growth, and all-cause mortality within 6 months. Success rate was 98.5 %. Total hospital stay was 3.17 ± 4 days. All-cause mortality was 8.9 %. Branch PA origin stenosis was found in 42 % cases on follow up. CONCLUSION:The study concludes that DS is a feasible and effective strategy in a variety of different cases of DDPC. Further research is needed to explore long-term outcomes and optimal stent selection based on individual patient characteristics.

A 62-year-old lady was referred with the diagnosis of hypertensive encephalopathy. She had episodes of paroxysms of hypertension while on the ventilator with normal saturations. She underwent a battery of tests to identify the cause of the paroxysms. Eventually, a very unusual cause was found that should have been identified if the label of hypertensive encephalopathy had not been put at the beginning. A diligent history and examination remain important even in the modern day with high-technology care.

A 6-month-old infant was operated on for supracardiac total anomalous pulmonary venous connection (TAPVC) with usual anatomy. The vertical vein was ligated. Weaning from bypass was attempted twice but was unsuccessful. Coronary sinus atresia was suspected and identified on the opening of the right atrium. A smooth postoperative course occurred after unroofing the coronary sinus. Coronary sinus atresia should be remembered as an uncommon association with TAPVC.

Sir, Significant diastolic flow reversal in the aortic arch is abnormal, and an important indicator of certain pathophysiological states. It denotes an aortic runoff proximal to the aortic arch as in aortic regurgitation, truncus arteriosus, aortopulmonary window, anomalous origin of the right or left pulmonary artery from the aorta, vein of Galen malformation, or other upper body systemic-to-pulmonary artery fistulae. It has also been described with severe pulmonary hypertension and a patent ductus arteriosus with inadequate left ventricular output as in patients with severe aortic stenosis, or aortic atresia, and in neonates with persistent pulmonary hypertension.[1-3] In a fetus, the retrograde flow in the aortic arch might be a poor prognostic sign.[4] Therefore, the presence of flow reversal in the aortic arch alerts the pediatric cardiologist to the possibilities of some definite abnormalities. We recently came across a 7-month-old child with transposition of great vessels, ventricular septal defect, patent ductus arteriosus, and pulmonary hypertension. The left and right ventricular function was normal. As the child was being evaluated for definitive surgery, he developed chest infection and worsened. A repeat echo showed a flow reversal in the aortic arch [Figure 1a and Supplementary Video 1], and therefore, further evaluation to rule out some of the disorders described above was done. The ultrasound of the cranium and a computed tomography angiogram of the brain did not show vein of Galen malformation. Furthermore, no systemic-to-pulmonary collateral or fistula was found. It was then recognized that the flow reversal is intermittent [Figure 1b and Supplementary Video 2] and was present when the child was sicker and hypoxic. The arterial saturation fluctuated from 58% (with reversal) to 77% (with no reversal). Even when the child cried, the reversal was present that disappeared subsequently.Figure 1: Suprasternal view of aortic arch color Doppler showing arch reversal (a), and no arch reversal (b) {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Supplementary Video 1","caption":"","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_6wtgaf7t"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Supplementary Video 2","caption":"","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_dk94y2p6"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} The exact cause of intermittent flow reversal in the aortic arch in our case is not clear. In previous reports of flow reversal with pulmonary hypertension and a ductus, the systemic ventricle output was compromised and the reversal was not intermittent. We hypothesize that it was possibly due to hypoxia and cerebral vasodilatation. Each time when the child was sicker, the flow reversal was found, and it disappeared when the saturations were better. We did not do arterial blood gas at that point in time, but the noninvasive saturation fluctuated significantly as described above. Why such a flow reversal is not more commonly seen with hypoxia remains speculative, nevertheless it seems the most plausible explanation for the intermittent runoff seen in this patient. Chronic hypoxia might be associated with hypocarbia that might balance the effects on cerebral blood flow. Aortic flow reversal with systemic hypercarbia has previously been described[5] but has not been widely recognized. In fact, even in the previous reports of flow reversal due to pulmonary hypertension, the patients with flow reversal were significantly hypoxic.[2,3] Awareness of this entity would avoid unnecessary investigations, and a reappraisal of flow reversal should be done in sick babies after correction of hypoxia or hypercarbia. We cannot ascertain with certainty whether other mechanistic reasons for flow reversal with crying are there or not, as detailed echo during crying was not possible. It is worthwhile to remember that the flow reversal in the aortic arch could be intermittent under some circumstances. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Myopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate. Severe pulmonary arterial hypertension has not been previously reported in patients with PUS1 gene mutation. A 17 year old girl with congenital sideroblastic anemia presented with worsening of breathlessness. Severe pulmonary artery hypertension was documented on investigations. A homozygous variant in exon 3 of gene PUS1,( chromosome 12:g.131932301 C > T c.430 C > T) was found on sanger sequencing. We document severe pulmonary arterial hypertension in a patient of congenital sideroblastic anemia from PUS1 gene. We hypothesis that cross talk with TGFb pathways might occur in PUS1 mutation, and that might cause severe PAH. This observation might have therapeutic implications.

Ventricular interdependence, i.e., reciprocal variations in the left and right ventricle pressures with respiration, is a hallmark of the hemodynamic diagnosis of constrictive pericarditis (CP). Similarly, respiratory variations in the mitral and tricuspid valve Doppler inflow velocities on echocardiogram are very helpful in the diagnosis of CP. We document the absence of such variations in a patient with CP and associated atrial septal defect. It is important to be mindful of this intuitively obvious fact; otherwise, the diagnosis of CP might be missed.

Arterial thoracic outlet syndrome (aTOS) is rarely diagnosed in children. A 15-year-old boy with recent-onset claudication was found to have aTOS on investigation. Bony abnormality other than the cervical rib might also cause aTOS. Enhanced awareness of aTOS as a cause of vascular symptoms in children is warranted.

AbstractBackgroundAcute rheumatic fever (ARF) and its chronic sequelae, rheumatic heart disease (RHD) contributes to valvular dysfunction and significant cardiovascular disability and endocardial damage is considered the primary pathophysiological mechanism underlying ARF. This study examined peripheral blood markers of endothelial injury and function in ARF and RHD patients and compared them to healthy controls.MethodIn this prospective observational study, the levels of collagen intermediates, matrix metalloproteinases, tissue inhibitors of matrix metalloproteinases, brain natriuretic peptide, Anti-DNaseB, VEGF, E-selectin, VCAM, and ICAM in circulation were estimated. The study also isolated hemangioblastic and monocytic endothelial progenitor cells and their respective microvesicles from the peripheral blood of patients and control samples.ResultsProcollagen type I carboxy-terminal propeptide, cross-linked c-telopeptide of type I, and procollagen III c-terminal propeptide levels were higher in RHD subjects compared to patients with ARF. The ARF patients had the highest levels of matrix metalloproteinases 10 (MMP-10) followed by chronic patients and healthy controls. The ratio of tissue inhibitors of matrix metalloproteinases TIMP-1 and MMP-10 was lowest in healthy controls.At the cellular level, there were higher number of monocytic endothelial progenitor cells (EPCs) in ARF subjects as compared to healthy controls. For hemangioblastic EPCs, there was no significant difference between chronic subjects and healthy controls, though their early subtype was higher in chronic subjects. The hemangioblastic EPCs microvesicles were more abundant in ARF compared to RHD patients.ConclusionThe greater number of EPCs and respective microvesicles confirm the continued disruption of the endothelium in ARF, and during the progression of the disease, the majority of EPCs undergo apoptosis.Obituary StatementThis study was conceived and designed by SR, PU, and Prof. Rajnish Juneja, Professor at AIIMS. Prof. Rajnish Juneja expired in April 2018 while the study was ongoing (1).Mr. Suran Nambisan, a research fellow at NII, was part of the team who initiated the experimental work. Dr. Suran Nambisan expired in January 2023.This paper is dedicated to both of them. It was the profound love for mankind and unwavering dedication to perfection by Prof. Rajnish Juneja that brought together a remarkable team to undertake this study. Dr. Suran Nambisan embarked on his professional research journey by successfully establishing and standardizing a few intricate protocols used in this study.

INTRODUCTION:Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making.MATERIALS AND METHODS:In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into "operable," "borderline," and "inoperable" based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision.RESULTS:298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m2 (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m2 (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m2, IQR 0.34) and borderline (0.80 ml/mmHg/m2, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m2 identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001).CONCLUSIONS:Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.

We hereby report rare occurrence of irreversible complete heart block in a child with tricuspid valve infective endocarditis. The tricuspid valve vegetation also caused complete closure of perimembranous ventricular septal defect, which was later discovered during surgery.