Congenital Cardiac Catheterization in Low- and Middle-Income Countries: The International Quality Improvement Collaborative Catheterization Registry.

Background: No published data are available on the patient, procedural characteristics, and outcomes of congenital heart disease (CHD) cardiac catheterization performed in low- and middle-income countries (LMICs). Objectives: The objective of this study was to describe procedural characteristics and patient outcomes of CHD cardiac catheterizations in LMICs. Methods: Cases performed between January 2019 and December 2020 from 15 centers in the International Quality Improvement Collaborative Congenital Heart Disease Catheterization Registry (IQIC-CHDCR) data were included. The Procedural Risk in Congenital Cardiac Catheterization (PREDIC3T) classification was used to stratify risk. Outcomes of interest included mortality, severe adverse events (SAEs), and procedural efficacy. Procedural efficacy, based on technical and safety endpoints, was categorized into optimal, adequate, and inadequate for 5 common interventional procedures. Results: There were 3,287 cases, of which 60% (n = 1,973) were interventional cases. Most of the cases (66%) were in patients between the ages of 1 to 18 years with a median patient age of 4 years. PREDIC3T risk class 1 and 2 were most common in 37% and 38% of cases, respectively. SAEs occurred in 2.8% while the death was reported within <72 hours post catheterization 1%. The majority of device implantation procedures patent ductus arteriosus (67%) and atrial septal defect (60%) had optimal procedure efficacy outcomes. Conclusions: This study demonstrates that congenital cardiac catheterization is safely performed in LMICs. Future work addressing predictors of SAEs and adverse procedural outcomes may help future quality improvement initiatives.

Cardiovascular Dysfunction in Children Exposed to Preeclampsia During Fetal Life.

BACKGROUND: Keeping in view the developmental origin of health and disease hypothesis, the aim of this study was to assess differences in cardiac and vascular structure and function in children exposed to preeclampsia in utero compared with those of normotensive mothers. The hypothesis under investigation was that children exposed to preeclampsia would have altered cardiac and vascular structure and function compared with the unexposed group. METHODS: This was a retrospective cohort study that included children 2 to 10 years of age born to mothers with and without exposure to preeclampsia in utero (n = 80 in each group). Myocardial morphology and function using echocardiography and carotid intima-media thickness and pulse-wave velocity were determined. Multivariate linear regression was used to compare preeclampsia-exposed and nonexposed groups. Subgroup analysis to assess differences between early- and late-onset preeclampsia was also performed. RESULTS: Forty-one percent of mothers (n = 33) had early-onset preeclampsia. Children in the exposed group had a significantly higher prevalence of stage 1 systolic and diastolic hypertension (22% [n = 18] and 35% [n = 18], respectively) compared with the unexposed group (9% [n = 7] and 19% [n = 15], respectively; P = .01). Children in the exposed group also had higher pulse-wave velocity compared with those in the unexposed group (0.42 ± 0.1 vs 0.39 ± 0.1, P = .03). Subgroup analysis revealed that changes in blood pressure and pulse-wave velocity were determined primarily by early-onset preeclampsia. There was no significant difference in cardiac morphology or systolic and diastolic function between the exposed and unexposed groups. CONCLUSION: In utero exposure to preeclampsia has an effect on vascular function in children aged 2 to 10 years, related primarily to early-onset disease. Routine blood pressure screening should be recommended for such children.

Adult congenital cardiac life-long needs evaluation in a low-middle income country, Pakistan.

OBJECTIVE: Adult congenital heart diseases (ACHD) have distinct health care needs that require life-long care. Limited data is available from low-middle income countries (LMIC). This descriptive study conducted in Pakistan, aimed to assess patients and health care professionals understanding of the needs for ACHD care and the perceived barriers to care. METHODS: A telephone survey was conducted of ACHD patients. An e mail survey was sent to the paediatric and adult cardiologists of five institutions (3 public and 2 private) that provide ACHD services in Pakistan. Descriptive statistics (frequencies, mean ± SD, median) were used for data analysis. RESULTS: A total of 128 ACHD patients were surveyed, 65 (51%) were females with a mean age of 29.4±10.4 years. Atrial septal defect repair was the most common surgical procedure. Mean age at surgery was 25.6±10.49 years, and a surgical follow-up period of 3.8±2.3 years. Majority (n=3, 60%) of the health care professionals (HCPs) responded that 75-100% of the ACHD surgical patients would need lifelong care, yet 10-25% return to their cardiology clinics. Most of the surveyed ACHD patients (89%, n=114) demonstrated a lack of understanding of life-long care after surgery due to not being communicated by their HCPs. Cost and travelling issues were the barriers highlighted by HCPs. Both ACHD patients (96%, n=122) and HCP (100%, n=5) underscored their interest in life long care. CONCLUSIONS: Majority of ACHD patients in Pakistan did not know that life-long follow-up is needed. Education regarding lifelong care for ACHD patients was identified as a means to alleviate the knowledge gap.

Health-related quality of life in congenital heart disease surgery in children and young adults: a systematic review and meta-analysis.

BACKGROUND: As survival improves in the congenital heart disease (CHD) population, health-related quality of life (HRQOL) outcomes become increasingly important. While surgery improves survival, poor HRQOL occurs postoperatively and cardiac-related HRQOL outcomes are rarely reported. OBJECTIVE: To conduct a systematic review and meta-analyses of general and cardiac-related HRQOL in CHD surgical children and young adults. METHOD: Medline, CINAHL and EMBASE were searched. Quantitative designs with a minimum of 80% CHD surgical patients and mean age ≤18 years compared with healthy controls were included in the review. Data were analysed in RevMan V.5.3 using a random effects model. OUTCOME MEASURES: General and cardiac-related HRQOL. RESULTS: Studies (n=20) were conducted in high-income countries and included 3808 patients plus 2951 parental reports of patients. HRQOL was worse in postoperative patients with CHD versus healthy controls in all domains with the largest difference seen for physical function (standard mean difference (SMD) of -0.56, 95% CI -0.82 to -0.30). Cardiac-related HRQOL was worse in complex compared with simple CHD with the largest SMD (-0.60, 95% CI -0.80 to -0.40) for symptoms. Heterogeneity ranged from 0% to 90%. CONCLUSIONS: CHD surgical patients have substantially worse HRQOL compared with age-matched healthy controls. Strategies should focus on improving HRQOL in this subgroup. Results may not be applicable to low/middle-income countries given the dearth of relevant research.

Impact of pre-eclampsia on the cardiovascular health of the offspring: a cohort study protocol.

INTRODUCTION: Pre-eclampsia is a common disorder associated with serious maternal and fetal complications. It is associated with abnormal placentation, which significantly reduces flow, resulting in a relative hypoxic state. These pathophysiological changes lead to subtle macrovascular and cardiac structural and functional changes in the fetus. This can predispose the child with maternal history of pre-eclampsia to risk of premature cardiovascular disease. METHODS AND ANALYSIS: The children will be identified from a cohort of women with pre-eclampsia. The study will be conducted at The Aga Khan University Hospital, Karachi. Inclusion criteria will be children who are between 2 and 5 years of age and have a maternal history of pre-eclampsia. The child's current weight, height and blood pressure will be recorded. A two-dimensional functional echocardiogram and vascular assessment will be performed to evaluate alterations in cardiac function as well as macrovascular remodelling in these children. Data will be presented as mean±SD, median (IQR) or percentages as appropriate. Independent t-test or Mann-Whitney U test will be used for testing of continuous variables (based on the assumption of normality). A p<0.05 will be used to determine statistical significance. ETHICS AND DISSEMINATION: Ethical approval has been obtained from AKUH Ethics Review Committee. Findings will be disseminated through scientific publications and project summaries for the participants.

Health-related quality of life in congenital heart disease surgery patients in Pakistan: protocol for a mixed-methods study.

INTRODUCTION: Reduced health-related quality of life (HRQOL) has been reported in postoperative patients with congenital heart disease (CHD). However, there is a paucity of data from low-income and middle-income countries (LMIC). Differences in sociodemographics and sociocultural contexts may influence HRQOL. This protocol paper describes a study exploring HRQOL in surgical patients with CHD from a tertiary hospital in Pakistan. The study findings will assist development of strategies to improve HRQOL in a resource-constrained context. METHODS AND ANALYSIS: This prospective, concurrent triangulation, mixed-methods study aims to compare HRQOL of postsurgery patients with CHD with age-matched healthy siblings and to identify HRQOL predictors. A qualitative component aims to further understand HRQOL data by exploring the experiences related to CHD surgery for patients and parents. Participants include patients with CHD (a minimum of n~95) with at least 1-year postsurgery follow-up and no chromosomal abnormality, their parents and age-matched, healthy siblings. PedsQL 4.0 Generic Core Scales, PedsQL Cognitive Functioning Scale and PedsQL 3.0 Cardiac Module will measure HRQOL. Clinical/surgical data will be retrieved from patients' medical files. Student's t-test will be used to compare the difference in the means of HRQOL between CHD and siblings. Multiple regression will identify HRQOL predictors. A subsample of enrolled patients (n~20) and parents (n~20) from the quantitative arm will be engaged in semistructured qualitative interviews, which will be analysed using directed content analysis. Anticipated challenges include patient recruitment due to irregular follow-up compliance. Translation of data collection tools to the Urdu language and back-translation of interviews increases the study complexity. ETHICS AND DISSEMINATION: Ethics approval has been obtained from The Aga Khan University, Pakistan (3737-Ped-ERC-15). Study findings will be published in peer-reviewed journals and presented at national and international conferences.

Conversion of cardiac bypass into an extracorporeal membrane oxygenation circuit: a case from Pakistan.

A 35 days old neonate with d-loop transposition of great arteries, underwent an arterial switch operation following which he developed hypotension attributed to left ventricular failure. During cardiopulmonary resuscitation decision was made to place him on cardiac bypass again. Due to limited resources and unavailability of a specialized extracorporeal membrane oxygenator machine, the CPB was modified and converted an ECMO. The neonate was successfully decannulated after 72 hours and discharged home after 3 weeks of the operation without any sequel. ECMO is a viable option in developing countries and may help in improving the outcome especially in neonatal congenital heart disease.

Outcome of infants with unrepaired heart disease admitted to the pediatric intensive care unit: single-center developing country perspective.

OBJECTIVE: Congenital heart disease (CHD) has an incidence of ∼0.8-1%. Outcome of previously diagnosed CHD patients awaiting surgery (either correction or palliation) in a developing country setting is unknown. We strive to determine the outcome of patients with CHD awaiting surgery who present to pediatric intensive care unit (PICU) setting with an acute illness. DESIGN: Retrospective cross-sectional chart review. SETTING: Pediatric intensive care unit of The Aga Khan University Hospital, Karachi, Pakistan. PATIENT: Medical records of infants (1-12 months) with CHD awaiting surgery presenting to the PICU with an acute illness between January 2009 and June 2012 were included. Newly diagnosed CHD patients, those not requiring PICU admission, and those transferred to another hospital were excluded. RESULTS: A total of 34 infants met the inclusion criteria. Median age at presentation was 5 months. Seventy-four percent of the infants had CHD lesion characterized by increased pulmonary blood flow (shunt lesions). Though none of the patients met the strict criteria for sepsis or pneumonia, 74% were admitted with a diagnosis of pneumonia or sepsis. Only 15% of patient had congestive heart failure as an admitting diagnosis. Oxygen therapy was given to 94% of these patients. Fifty-nine percent of these patients expired during the admission, 95% of those expired had multiorgan dysfunction. CONCLUSION: Patients with CHD awaiting surgery and who admitted to the PICU with acute illness are at high risk for mortality. Stringent criteria to diagnose pneumonia or sepsis should be used in these patients.

Management of acute myocarditis in children.

Myocarditis is defined as the inflammation of the myocardium. It continues to be a significant cause of morbidity and mortality in the paediatric population and is the commonest cause of cardiac failure in a healthy child. Some studies estimate the incidence of myocarditis to be around 1 per 100 000. PubMed search was performed using the term 'myocarditis.' The search was limited to age 0-19 years. A total of 50 articles were identified between 1966 to date and reviewed. Myocarditis is a challenging diagnosis to make on clinical grounds and requires high index of suspicion. The cornerstone of treatment remains supportive though therapeutic modalities such as immunosuppressive and intravenous immunoglobulin therapies are being studied extensively. The overall prognosis of the disease is good with survival rates up to 80%.

Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum.

OBJECTIVE: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). BACKGROUND: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. METHODS: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. RESULTS: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. CONCLUSIONS: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Exercise stress echocardiographic assessment of outflow tract and ventricular function in patients with an obstructed right ventricular-to-pulmonary artery conduit after repair of conotruncal heart defects.

This study assessed right ventricular (RV) and RV outflow tract (RVOT) function and pressure in response to exercise in patients with an obstructed RV-pulmonary artery (PA) conduit using exercise stress echocardiography (ESE) to evaluate these parameters. RV-PA conduits inevitably develop stenosis and/or regurgitation over time. Assessment of conduit obstruction only at rest may not reveal the extent of physiologic perturbation related to RV pressure loading. Patients with a stenotic RV-PA conduit who were being considered for transcatheter pulmonary valve placement were approached prospectively. ESE was performed and ventricular images were obtained at rest and at peak exercise. Forty patients (median age 17 years) were enrolled. Most patients had tetralogy of Fallot (63%) and were in New York Heart Association class II (59%). Exercise stress echocardiographic images were adequate in 38 patients (95%). With exercise there was a significant increase in maximum instantaneous RVOT gradient from rest (59 vs 96 mm Hg, p <0.001); exercise-induced change in RVOT gradient correlated with global RV strain at rest (r = -0.3, p = 0.05). Compared to measurements at rest there were significant increases in median peak longitudinal strain of the left ventricular free wall, interventricular septum, and global left ventricular strain at peak exercise. There were no significant changes in median RV strain at peak exercise (RV free wall -14.3 [-26, -8] at rest vs -15.2 [-27, -3] at peak exercise, p = 0.87; global RV strain -13.9 [-32, -9] vs -15.1 [-23, -6], p = 0.11). In conclusion, using ESE it was possible to evaluate abnormal ventricular function and conduit dysfunction at peak exercise in patients with an obstructed RV-PA conduit.

Postnatal angiographic appearance of left ventricular myocardium in fetal patients with aortic stenosis having in-utero aortic valvuloplasty.

Fetal aortic valvuloplasty (FAV) is performed on the basis of the hypothesis that aortic stenosis leads to the impairment of left ventricular (LV) filling and growth. Given that most fetuses fail to exhibit normalization of LV growth even after successful FAV, better understanding of the associated LV myocardial pathology is indicated. Postnatal angiography was reviewed retrospectively for all patients who (1) underwent FAV for severe aortic stenosis and (2) had well-opacified LV angiograms before any surgical intervention from 2000 to 2007. The angiographic appearance of the LV myocardium was described as either smooth or trabeculated in a total of 6 anatomic segments in 2 projections (anteroposterior and lateral). Twenty-four infants who underwent FAV had angiographic images appropriate for review. Of these, 4 (17%) had uniformly smooth LV myocardium, whereas 20 (83%) had deep trabeculations of > or =1 LV myocardial segment. Half of the infants (n = 13) had extensive trabeculations with the involvement of > or =4 segments. In all cases, the septal segments were smooth. In conclusion, patients after FAV have angiographically abnormal left ventricles, commonly involving extensive trabeculations of the free wall, but without septal involvement.

Somatic growth after fontan and mustard palliation.

OBJECTIVE: Patients with complex congenital heart disease frequently develop early growth failure; however, the long-term outcome for growth after surgery for single ventricle or anatomic right ventricle as systemic ventricle is not clear. This study was designed to determine long-term growth in patients following the Fontan and Mustard operations. METHOD: We retrospectively reviewed the growth parameters of children who had previously undergone the Fontan (n = 80) or Mustard (n = 66) palliation at the Riley Hospital for Children, Indiana. RESULTS: Both the Fontan and Mustard groups had normal height and weight at birth. At the time of their Fontan or Mustard palliation, there was a significant retardation in weight (Z-score: -0.98 and -1.79, respectively) and height (Z-score: -0.96 and -1.03, respectively). Both cohorts postoperatively demonstrated significant catch-up in their weights. Although the Mustard group normalized their heights, the Fontan patients continued to demonstrate short statures in long-term follow-up. CONCLUSION: Children with single ventricles and those with palliated d-loop transposition of the great arteries suffer somatic growth delay prior to definitive surgery, despite being of normal size at birth. Catch-up growth in weight occurs after the Fontan and Mustard operations. In the Mustard population, height also normalizes, whereas in patients with univentricular circulation, height remains abnormally low.