Impact of rural residence and low socioeconomic status on rate and timing of prenatal detection of major congenital heart disease in a jurisdiction of universal health coverage.

OBJECTIVE: Socioeconomic status (SES) and distance of residence from tertiary care may impact fetal detection of congenital heart disease (CHD), partly through reduced access to and quality of obstetric ultrasound screening. It is unknown whether SES and remoteness of residence (RoR) affect prenatal detection of CHD in jurisdictions with universal health coverage. We examined the impact of SES and RoR on the rate and timing of prenatal diagnosis of major CHD within the province of Alberta in Canada. METHODS: In this retrospective study, we identified all fetuses and infants diagnosed with major CHD in Alberta, from 2008 to 2018, that underwent cardiac surgical intervention within the first year after birth, died preoperatively, were stillborn or underwent termination. Using maternal residence postal code and geocoding, Chan SES index quintile, geographic distance from a tertiary-care fetal cardiology center and the Canadian Index of Remoteness (IoR) were calculated. Outcome measures included rates of prenatal diagnosis and diagnosis after 22 weeks' gestation. Risk ratios (RR) were calculated using log-binomial regression and stratified by rural (≥ 100 km from tertiary care) or metropolitan (< 100 km from tertiary care) residence, adjusting for year of birth and the obstetric ultrasound screening view in which CHD would most likely be detected (four-chamber view; outflow-tract view; three-vessel or three-vessels-and-trachea or non-standard view; septal view). RESULTS: Of 1405 fetuses/infants with major CHD, prenatal diagnosis occurred in 814 (57.9%). Residence ≥ 100 km from tertiary care (adjusted RR, 1.19; 95% CI, 1.05-1.34) and higher IoR (adjusted RR, 1.9; 95% CI, 1.1-3.3) were associated with missed prenatal diagnosis of major CHD. Similarly, residence ≥ 100 km from tertiary care (adjusted RR, 1.41; 95% CI, 1.22-1.62) and higher IoR (adjusted RR, 3.6; 95% CI, 2.2-8.2) were associated with prenatal diagnosis after 22 weeks. Although adjusted and unadjusted analyses showed no association between Chan SES index quintile and prenatal-diagnosis rate overall nor for residence in rural areas, in metropolitan regions, lower SES quintiles were associated with missed prenatal diagnosis (quintile 1: RR, 1.24; 95% CI, 1.02-1.50) and higher risk of diagnosis after 22 weeks' gestation (quintile 1: RR, 1.46; 95% CI, 1.10-1.93; quintile 2: RR, 1.66; 95% CI, 1.24-2.23). CONCLUSIONS: Despite universal healthcare, rural residence in Alberta is associated with lower rate of prenatal diagnosis of major CHD and higher risk of late prenatal diagnosis (≥ 22 weeks). Within metropolitan regions, lower SES impacts negatively prenatal-diagnosis rate and timing. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Clinical presentation, genetic etiology and outcome associated with fetal cardiomyopathy: comparison of two eras.

OBJECTIVE: To assess the rate and type of genetic diagnosis and clinical outcome of cases of fetal cardiomyopathy (CM) during two eras, in order to examine the impact of advanced genetic testing and improved perinatal management strategies. METHODS: All diagnoses of fetal CM in Alberta, Canada, encountered between 2003 and 2019, were reviewed retrospectively. Genetic, cardiac and non-cardiac diagnoses and clinical outcome were documented. Cases with CM secondary to extracardiac pathology and maternal conditions were excluded. Cases diagnosed in the earlier era of the study period (2003-2012) were compared with those diagnosed in the later era (2013-2019). RESULTS: Thirty-eight cases of fetal CM met the inclusion criteria. Median gestational age (GA) at diagnosis was 22.8 (range, 13.4-37.4) weeks. Associated structural heart disease was present in 39% (15/38) of cases and 24% (9/38) had arrhythmia. Hydrops was identified in 29% (11/38) of cases at presentation, and a further 18% (7/38) developed hydrops later in gestation. Twenty-six percent (10/38) of cases underwent termination of pregnancy and 24% (9/38) had intrauterine death. Of liveborn cases, neonatal death occurred in 16% (3/19), late death occurred in 21% (4/19) and 63% (12/19) were alive at the last follow-up. Excluding cases that had termination of pregnancy and those with a liveborn infant who received planned palliative care, the rate of neonatal survival was higher in the later compared with the earlier era (69% (11/16) vs 45% (5/11)), although the difference was not statistically significant (P = 0.26). A genetic etiology was confirmed in 39% (15/38) of cases and strongly suspected in 24% (9/38). A significantly higher proportion of cases had a confirmed or strongly suspected genetic etiology in the later era compared with in the earlier era (76% (19/25) vs 38% (5/13); P = 0.04). CONCLUSIONS: In the recent era, a higher proportion of fetal CM cases had a confirmed or strongly suspected genetic etiology than reported previously. Based on comparison with older series, modern perinatal management strategies may not have a significant impact on neonatal survival in cases of fetal CM; however, a larger study would be better powered to detect more subtle differences. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Fetal echocardiographic predictors of biventricular circulation in hypoplastic left heart complex.

OBJECTIVES: To determine which echocardiographic features of hypoplastic left heart complex (HLHC) in the fetal period are predictive of biventricular (BV) circulation and to evaluate the long-term outcome of patients with HLHC, including rates of mortality, reintervention and development of further cardiac disease. METHODS: Echocardiograms of fetuses with HLHC obtained at 18-26 weeks and 27-36 weeks' gestation between 2004 and 2017 were included in the analysis. The primary outcome was successful BV circulation (Group 1). Group 2 included patients with single-ventricle palliation, death or transplant. Univariate analysis was performed on data obtained at 18-26 and 27-36 weeks and multivariate logistic regression was performed on data obtained at 27-36 weeks only. RESULTS: Of the 51 included cases, 44 achieved successful BV circulation (Group 1) and seven did not (Group 2). Right-to-left/bidirectional foramen ovale (FO) flow and a higher mitral valve (MV) annulus Z-score were associated with successful BV circulation on both univariate and multivariate analysis. Bidirectional or left-to-right FO flow, left ventricular length (LVL) Z-score of < -2.4 and a MV Z-score of < -4.5 correctly predicted 80% of Group 2 cases. Late follow-up was available for 41 patients. There were two late deaths in Group 2. Thirteen patients in Group 1 required reintervention, 12 developed mitral stenosis and five developed isolated subaortic stenosis. CONCLUSIONS: BV circulation is common in fetuses with HLHC. Higher MV annulus and LVL Z-scores and right to left direction of FO flow are important predictors of BV circulation. Long-term sequelae in those with BV circulation may include mitral and subaortic stenosis. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Population trends in prenatal detection of transposition of great arteries: impact of obstetric screening ultrasound guidelines.

OBJECTIVES: Dextro-transposition of the great arteries (d-TGA) is one of the most common critical neonatal heart defects, with a low detection rate antenatally. We sought to evaluate trends in the prenatal detection of d-TGA with or without ventricular septal defect (VSD) in Alberta over the past 13 years, examining the potential impact of ultrasound guidelines incorporating screening of cardiac outflow tracts, updated in 2009-2010 and in 2013, and factors affecting detection of the condition. METHODS: All fetuses and neonates with d-TGA, with or without VSD, encountered between 2003 and 2015 in the province of Alberta, were identified retrospectively. Clinical records including obstetric ultrasound reports were reviewed. Pregnancy outcome, common referral indications and associated maternal and fetal pathology in affected pregnancies were assessed. RESULTS: From 2003 to 2015, 127 cases with d-TGA were encountered in Alberta, of which 47 (37%) were detected prenatally. Prenatal detection improved over the study period, from 14% in 2003-2010, to 50% in 2011-2013, and to 77% in 2014-2015. Of the 47 fetuses with a prenatal diagnosis of d-TGA, an indication for fetal echocardiography included abnormal or poorly visualized cardiac outflows with normal four-chamber view in 46 (98%). Comorbidities were identified in 12 mothers, only five of which represented an additional reason for fetal echocardiography referral, and four fetuses had extracardiac pathology. CONCLUSION: Substantial improvement in the prenatal detection of d-TGA has been observed in Alberta over the past few years, owing to improved screening of cardiac outflow tracts on routine obstetric ultrasound examination in otherwise healthy pregnancies, and has been temporally associated with updated obstetric ultrasound guidelines suggesting that these contributed to optimized screening of affected pregnancies. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Stenting of the right ventricular outflow tract in the symptomatic infant with tetralogy of Fallot.

BACKGROUND: Repair of neonatal tetralogy of Fallot (TOF) has low mortality. Debate continues regarding the initial management of cyanotic or duct-dependent infants with TOF and adverse risk factors. While repair can and has been performed in these patients, it is associated with increased morbidity. OBJECTIVE: We review the effectiveness of right ventricular outflow tract (RVOT) stenting in the symptomatic young infant with TOF. METHODS: Clinical, echocardiographic, angiographic and haemodynamic data were reviewed for nine patients who underwent 11 RVOT stenting procedures from October 1994 to August 2007. RESULTS: The pulmonary valve was deemed unsalvageable in all patients (median valve diameter 3.7 mm (range 2.7-4.2), Z-score -6.7 (range -9.7 to -5.4). RVOT stenting improved arterial oxygen saturation from a median of 73% (60-85%) to 94% (90-98%) (p = 0.008). Median Z-score for the left pulmonary artery increased from -4.9 (-7.8 to -2.4) before stent implantation to -1.5 (-4.2 to -0.2) (p = 0.02) before surgical repair. Median Z-score for the right pulmonary artery increased from -3.7 (-6.8 to -1.9) to -0.8 (-2.5 to 0.1) (p = 0.008). Median Nakata index increased from 56 mm(2)/m(2) (21-77) to 150 mm(2)/m(2) (123-231) (p = 0.008). There were no procedural complications. Six patients have undergone successful repair. There were no deaths. CONCLUSIONS: In the symptomatic young infant with TOF, stenting of the RVOT provides a safe and effective management strategy, improving arterial oxygen saturation and encouraging pulmonary artery growth.

Hypoplastic left heart syndrome: Prognosis and management options.

Few congenital heart malformations have raised as many surgical, ethical, social and economic issues as the therapy for infants diagnosed with hypoplastic left heart syndrome. Before the 1980s, this complex malformation was associated with 95% mortality within the first month of life. In the past two decades, palliative surgery and cardiac transplantation have become management options, in addition to comfort care for the infant. These innovations have forced parents and physicians to make difficult decisions because the long term results of the additional treatment options are not known. This article describes the current risk factors, diagnosis, treatment and outcome of infants with hypoplastic left heart syndrome. Prenatal diagnosis provides families with time for counselling and for becoming more informed about management options. Surgical therapy provides hope for the survival of these infants, but their long term outcomes are not well defined. Comfort care in either the home or hospital remains an acceptable management option. More investigations to determine the long term outcome following palliative surgery and transplantation are needed before they become the standards of care.

Cardiac surgery in octogenarians: can elderly patients benefit? Quality of life after cardiac surgery.

BACKGROUND: Increasing numbers of the very old are presenting for cardiac surgical procedures. There is little information about quality of life after hospital discharge in this group. METHODS: From March 1995 to February 1997, 127 patients older than 80 years at operation (mean age, 83+/-2.5 years; range, 80 to 92 years) were entered into the cardiac surgery database and analyzed retrospectively. The RAND SF-36 Health Survey and the Seattle Angina Questionnaire were used to assess quality of life by telephone interview (mean follow-up, 15.7+/-6.9 months). No patient was lost to follow-up. RESULTS: Operations included coronary artery bypass grafting (65.4%), coronary artery bypass grafting plus valve replacement (15.8%), and isolated valve replacement (14.2%). Preoperatively, 63.8% were in New York Heart Association class IV. Thirty-day mortality was 7.9%, and actuarial survival was 83% (70% confidence interval, 79% to 87%) at 1 year and 80% (70% confidence interval, 75% to 85%) at 2 years. Preoperative renal failure significantly increased the risk of early death (relative risk, 3.96) as did urgent or emergent operation (relative risk, 6.70). In addition, cerebrovascular disease (relative risk, 3.54) and prolonged ventilation (relative risk, 3.82) were risk factors for late death. Ninety-five patients (92.2%) were in New York Heart Association class I or II at follow-up. Seattle Angina Questionnaire scores for anginal frequency (92.3+/-18.9), stability (94.4+/-16.5), and exertional capacity (86.8+/-25.1) indicated good relief of symptoms. SF-36 scores were equal to or better than those for the general population of age greater than 65 years. Of the survivors, 83.7% were living in their own home, 74.8% rated their health as good or excellent, and 82.5% would undergo operation again in retrospect. CONCLUSION: Octogenarians can undergo cardiac surgical procedures at a reasonable risk and show remarkable improvement in their symptoms. Elderly patients benefit from improved functional status and quality of life.