Pulmonary arterial compliance in patients of CHD with increased pulmonary blood flow.

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.

A rare pediatric case of bilateral bronchopulmonary vascular malformations and right isomerism.

We present an unusual case of an 18-day-old term neonate with coexistent bilateral bronchopulmonary vascular malformations and right isomerism. This case highlights the importance of computed tomography angiography in depicting such complex anomalies and classifying them according to components involved providing a systematic approach for evaluation of the disease process.

Effect of acute lower respiratory tract infection on pulmonary artery pressure in children with post-tricuspid left-to-right shunt.

We sought to examine the influence of clinically severe lower respiratory tract infection on pulmonary artery pressure in children having CHD with post-tricuspid left-to-right shunt, as it may have physiological and clinical implications. In a prospective single-centre observational study, 45 children with post-tricuspid left-to-right shunt and clinically severe lower respiratory tract infection were evaluated during the illness and 2 weeks after its resolution. Pulmonary artery systolic pressure was estimated non-invasively using shunt gradient by echocardiography and systolic blood pressure measured non-invasively.Median pulmonary artery systolic pressure during lower respiratory tract infection was only mildly (although statistically significantly) elevated during lower respiratory tract infection [60 (42-74) versus 53 (40-73) mmHg, (p < 0.0001)]. However, clinically significant change in pulmonary artery systolic pressure defined as the increase of >10 mmHg was present in only 9 (20%) patients. In the absence of hypoxia or acidosis, only a small minority (9%, n = 4) showed significant pulmonary artery systolic pressure rise >10 mmHg. In the absence of hypoxia or acidosis, severe lower respiratory tract infection in patients with acyanotic CHD results in only mild elevation of pulmonary artery systolic pressure in most of the patients.

Characteristics of Children with Acute Rheumatic Carditis from a High-Incidence Region: Importance of Unexplained Worsening of Functional Class.

BACKGROUND: Acute rheumatic fever (ARF) and acute rheumatic carditis (ARC) continue to be a major public health problem in developing countries. OBJECTIVE: To study the characteristics of children with ARC being treated at a tertiary centre. METHODS AND RESULTS: We studied 126 children (mean age 10.4 ± 2.3 years, range 5-15 years, 60% males) diagnosed with ARC by treating cardiologists. Most had lower socio-economic status. Fifty of 126 (40%) presented with a first episode of ARC. Joint symptoms were present in 29% and fever in 25%. Only 2.4% had subcutaneous nodules and none had erythema marginatum or chorea. Fifty-one percent presented in NYHA class II and 29% in NYHA class III or IV. Tachycardia and heart failure were present in 53% and 21%, respectively. Recent worsening of NYHA class (dyspnoea) was the commonest feature (48%). Laboratory investigations showed raised antistreptolysin O titres (>333 units) in only 36.7% of patients. Raised C-reactive protein (CRP) was present in 70%, while raised erythrocyte sedimentation rate was found in only 37% of patients. On the basis of above findings, the modified Jones criteria (2015) for the diagnosis of ARF were satisfied only in 46% of children. Echocardiography showed mitral valve thickening in 77% and small nodules on the tip of the leaflets in 43% (27 and 8%, respectively for aortic valve). Left ventricular ejection fraction was <50% in only 3 patients. The dominant valve lesion was mitral regurgitation (MR) (present in 95% of patients; severe in 78%, moderate in 15%), while aortic regurgitation was present in 44% (severe in 14%). CONCLUSIONS: The criteria are often not satisfied by patients being treated for ARC. Recent unexplained worsening of dyspnoea, young age, significant MR, echocardiographic nodules, and elevated CRP are important indicators.

Separate arch origin of the left external carotid artery with common trunk giving rise to the left internal carotid artery and left subclavian artery.

We present a case of a 3-year-old boy with tetralogy of Fallot having the direct origin of the left external carotid artery from the aorta with a common trunk giving rise to the left internal carotid artery and left subclavian artery, in a right-sided aortic arch. We also highlight the potential implications in management.

Operable ventricular septal defect despite severe pulmonary hypertension and cyanosis!

Patients with a significant left-to-right shunt at ventricular level may become inoperable at an early age due to irreversible pulmonary vascular disease. On the other hand, even suprasystemic pulmonary hypertension due to mitral stenosis remains treatable. We report a 24-year-old patient with large ventricular septal defect, severe mitral stenosis and cyanosis who improved after surgical correction of both the lesions. This emphasises the importance of additional post-capillary pulmonary hypertension in Eisenmenger syndrome.

False positive retroaortic left circumflex coronary artery in a patient with atrial septal defect.

Retroaortic course of coronary artery is a relative contraindication for device closure of an atrial septal defect. In this brief report, we demonstrate, for the first time, inferior aortic recess mimicking retroaortic left circumflex coronary artery in a patient with atrial septal defect. This distinction is important to avoid spurious diagnosis of anomalous coronary artery denying patient a chance of nonsurgical closure of atrial septal defect.

Operable patent ductus arteriosus even with differential cyanosis: a case of patent ductus arteriosus and mitral stenosis.

Patients with patent ductus arteriosus and significant left-to-right shunt develop Eisenmenger syndrome at an early age and are not operable after development of irreversible pulmonary artery hypertension. Patients with mitral stenosis, however, are treatable even with suprasystemic pulmonary artery pressures. A combination of these two lesions is rare. We document a patient with differential cyanosis who improved after corrective surgery of both the lesions. The importance of post-capillary pulmonary artery hypertension in shunt lesions needs to be better appreciated.

Perforating the atretic pulmonary valve with CTO hardware: Technical aspects.

OBJECTIVES: To review the success and technical aspects of pulmonary valve (PV) perforation using chronic total occlusion (CTO) hardware in patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Interventional therapy is possible in selected patients with PA-IVS. Among the various interventional options available, radiofrequency and laser assisted perforation may be more successful, but require expertise and may be substantially costly. METHODS: We describe the technique of mechanical catheter PV perforation using currently available coronary hardware meant for coronary CTO in nine cases with PA-IVS. After complete echocardiographic evaluation and informed parental consent was obtained, patients were electively intubated, mechanically ventilated, adequately heparinized and were placed on intravenous prostaglandin infusion. Basic steps involved were-localizing the atretic segment and accomplishing coaxial alignment of catheters using biplane fluoroscopy, crossing the atretic segment with the soft end of perforating guidewire, stabilizing the assembly and performing graded balloon dilatation with the balloon size never exceeding 130% of pulmonary annulus diameter. For crossing the atretic PV, a retrograde approach was used in one patient where the antegrade approach was not possible. RESULTS: The procedure was successful in 8/9 cases (89%). Valve opening was achieved in all eight patients with immediate fall in right ventricular (RV) systolic pressures. One neonate died following surgery after catheter induced RV perforation. All surviving cases were discharged from the hospital in good general condition with no evidence of heart failure and a room air oxygen saturation of >85%. No patient required an additional pulmonary irrigation procedure. CONCLUSION: With appropriate patient and hardware selection, PV perforation using readily available coronary hardware is feasible in PA-IVS. © 2014 Wiley Periodicals, Inc.

Changes in Myocardial Contractility and Electromechanical Interval During the First Month of Life in Healthy Neonates.

This study aims at documenting the changes in ventricular tissue velocities, longitudinal strain and electromechanical coupling during the first month of life. During the neonatal period, when the ventricular myocardium is not yet fully maturated, the heart is subjected to significant hemodynamic changes. We studied the ventricular performance of 16 healthy neonates at three time points over the first month of life: on days 2 (IQR [2;2]), 13 [12;14] and 27 [25;29]. We found that systolic and diastolic tissue velocities increased significantly in both left and right ventricle (by 1.2-1.7 times, p < 0.001). Congruently, we found that peak systolic longitudinal strain of the right and left ventricles increased significantly. However, no significant changes in longitudinal strain rate were observed. Finally, QS-intervals shortened during the neonatal period: being measured at 12 points throughout the left ventricle, time to peak systolic velocity decreased on average to 89 % in the second and to 80 % in the fourth week of life (22.3 ± 0.2 vs. 19.8 ± 0.3 vs. 17.8 ± 0.5 ms, r = -0.564, p < 0.001). When comparing opposing walls of the left ventricle, no dyssynchrony in left ventricular contraction was found. In addition to increasing systolic and diastolic tissue velocities during the first month of life, the time to peak systolic contraction shortens in the neonatal heart, which may reflect an increasing efficiency of the excitation-contraction coupling in the maturing myocardium. While there appears to be no dyssynchrony in ventricular contraction, these findings may extend our appreciation of the immature neonatal heart and certain disease states.

Saline Contrast Echocardiography in the Era of Multimodality Imaging--Importance of "Bubbling It Right".

Saline contrast echocardiography is an established imaging modality. Logical interpretation of a carefully performed study is vital to realize its diagnostic potential. In this review, we discuss utility of saline contrast echocardiography in evaluation of various pathologies within and outside the heart other than a patent foramen ovale.

Pulmonary atresia and intact ventricular septum with transposed arterial trunks.

We report a case of pulmonary atresia with intact ventricular septum, but in the setting of transposed great arteries, and thus the left rather than the right ventricle was hypoplastic.

Hemodynamics of large ventricular septal defect and coexisting chronic constrictive pericarditis masquerading as Eisenmenger's syndrome.

The presence of right to left shunting due to elevated pulmonary vascular resistance (PVR) in a patient with large ventricular septal defect (VSD) is conventionally considered as Eisenmenger's syndrome. The recognition of reversible causes of elevated PVR is important. We report hemodynamics of a child in whom unusual coexistence of large VSD and chronic constrictive pericarditis (CCP) mimicked Eisenmeneger's syndrome. The strategy of stepwise hemodynamic evaluation led to good clinical outcome.

De novo fenestration of extra-cardiac Fontan Goretex conduit assisted by Inoue balloon.

De novo fenestration of Goretex conduit of extra-cardiac total cavopulmonary connection in the postoperative period is challenging, and is rarely reported. We report a 17-year-old boy with failing Fontan circuit in whom fenestration was created, aided by an Inoue balloon.

Inflammatory markers are elevated in Eisenmenger syndrome.

Inflammation may be an important contributing factor to the progression of Eisenmenger syndrome (ES). Markers of systemic inflammation in ES have not been systematically studied. Inflammatory markers including high-sensitivity C-reactive protein (hs-CRP), interleukin-2 (IL-2), IL-6, and interferon-γ (IFN-γ) were measured in 42 consecutive ES patients (mean age, 24.3 ± 10.6 years) compared with their levels in 22 healthy control subjects. The patients were followed up for a mean duration of 16.3 ± 13.7 months. The levels of inflammatory markers were correlated with clinical and hemodynamic variables at baseline and the outcomes of death, hospitalization, and worsening World Health Organization (WHO) functional class at follow-up evaluation. Compared with the control subjects, ES patients showed a significant elevation in hs-CRP (2.99 ± 3.5 vs 1.1 ± 0.9 mg/dl; p = 0.002) and IFN-γ (41.3 ± 43.6 vs 10.4 ± 6.9 pg/ml; p < 0.001) levels. The levels of IL-2 and IL-6 also were elevated but did not differ significantly from those in the control subjects. The patients with hs-CRP levels higher than 3 mg/dl were significantly older (28.9 ± 10.6 vs 21.5 ± 9.8 years) and had a significantly shorter 6-min walk distance (421.5 ± 133.2 vs 493.3 ± 74.8 m). The levels of inflammatory markers did not correlate with baseline parameters or clinical outcomes. To conclude, the levels of hs-CRP and IFN-γ are significantly elevated in ES. Elevated hs-CRP in ES was associated with older age and shorter 6-min walk distance, but the levels of inflammatory markers were not predictive of clinical events.

Arterial thoracic outlet syndrome in an adolescent.

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.

Lack of "ventricular interdependence" in constrictive pericarditis and atrial septal defect.

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.

Failure to wean off after a routine total anomalous pulmonary venous connection operation: An uncommon association.

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.