What Is the Survival Rate of a Baby Born Woth 2 Atreries in the Heart

Introduction

Congenital centre affliction (CHD) is a range of structural anomalies of the heart, which affect around 1% of births.^ane Individuals with CHD oft require complex life‐saving surgeries in infancy and lifetime follow‐up.² Survival estimates are therefore important to understand prognosis and evaluate health and social intendance needs. Our recent systematic review of sixteen population‐based studies estimated a 5‐year survival of 85%, ranging from 14% for hypoplastic left heart to 96% for ventricular septal defect.³ CHD survival is strongly influenced by gestational historic period at birth, birth weight, and the presence of extracardiac anomalies (ie, additional congenital anomalies outside the cardiovascular system),^{4 , v , six , 7 , 8 , 9} but there is conflicting evidence for plurality, maternal age, ethnicity, and socioeconomic impecuniousness.^{4 , 5 , 6 , seven , 8 , 9 , 10 , 11} Previous studies of CHD mortality tend to written report adventure ratios (HRs) without reporting survival estimates, which have greater utility for counseling and service planning.

The aim of this study is to estimate long‐term survival of individuals with CHD, born in the North of England during 1985–2003, conditioned on important determinants.

Methods

Case Inclusion

The Northern Congenital Abnormality Survey (NorCAS) is a population‐based register that collects information on cases of congenital anomalies delivered to women residing in the Due north of England (Effigy). Cases diagnosed upwardly to age xvi years (historic period 12 years afterwards 2001) are notified from multiple sources, including prenatal ultrasound, fetal medicine, cytogenetic laboratories, the regional cardiology center, pathology and paediatric surgery, and coded using the World Health Organization International Classification of Diseases.

• Download PowerPoint

Effigy one. Map showing the area covered by the Northern Congenital Aberration Survey.

Cases with CHD (International Classification of Diseases, Tenth Revision: Q20–26) live born betwixt Jan i, 1985 and December 31, 2003 were included. Cases with a single small CHD, for example, patent ductus arteriosus born <37 weeks' gestation, were excluded as per European Surveillance of Congenital Anomalies guidelines.¹²

Survival Condition

Deaths before historic period 1 year were identified from the region'due south Perinatal Mortality Survey, which is linked to the NorCAS through the mother's details. The Perinatal Bloodshed Survey collects data on infant deaths from statutory death registrations for infants whose mothers reside in the North of England (Figure). Deaths beyond age 1 twelvemonth were identified by linking with national death registrations from the Office for National Statistics. The linkage was performed on January 28, 2008 using "fuzzy matching" by infant proper name, last known accost, female parent's age at delivery and sex activity. Cases not on the Perinatal Bloodshed Survey or matched with Office for National Statistics records (eg, nascence certificates) were cross‐referenced with the NorCAS or hospital records and located through the National Health Service National Strategic Tracing Service. Those that could not exist traced were excluded (0.five%). Cases with matched death registrations were classified as deceased on their date of death; those without were coded as live and censored on the date the linkage was performed.

Ethical Approval

The NorCAS has approval from the Confidentiality Informational Group of the Wellness Enquiry Authority (PIAG 2‐08(e)/2002) to agree data without consent and, at the time of study, ideals approval (09/H0405/48) to undertake research involving the information. This written report was given a favorable ethical opinion by the South Tees Local Enquiry Ethics Commission.

Information

Using a fetal growth formula, nativity weight at xl weeks gestational age was predicted for all cases (based on their bodily birth weight and gestational age).¹³ Regional nascence weight references were applied to this formula, with standardization for gestational age at birth, sexual practice, and plurality.^{13 , 14}

From mothers' residential postcode at delivery, the Index of Multiple Deprivation 2004 was calculated. Alphabetize of Multiple Impecuniousness is a mensurate of area‐level socioeconomic deprivation calculated from income, employment, health, teaching, access to services, social environment, housing stress, living environment, and offense.^{xv , 16} Alphabetize of Multiple Deprivation rank, which ranges from ane for the most deprived area to 32 844 for the least deprived area, was categorized as least, boilerplate, and nearly deprived tertiles. The tertiles were created regionally to allow virtually equal numbers of cases to be assigned to each category.

The following were investigated as adventure factors of mortality: year of nascency (continuous); maternal historic period at birth (continuous); extracardiac anomalies (none, structural, or chromosomal/genetic); standardized nascence weight (low [z <−i], average [−1≤ z ≤1], high [z >1]); gestational historic period at birth (very preterm [<32 weeks]; moderate preterm [32–36 weeks]; term [37–41 weeks]; post‐term [≥42 weeks]); socioeconomic impecuniousness (least, boilerplate, or most deprived), infant sex (male or female person); and plurality (singleton or twin). CHD severity was coded into 4 groups (I [most severe], II, III [to the lowest degree severe], and "unclassified"), using a slightly adapted version of Khoshnood et al'southward coding organization.¹⁷ Unclassified CHD includes patent ductus (≥37 weeks), congenital centre block, dextrocardia, and aortic regurgitation, amongst others. Cases with multiple subtypes were coded according to the virtually astringent and thus may vary from those previously reported from these data.^eighteen Table 1 shows which CHD subtypes are in each severity category.

Table 1. Survival for All CHD According to Risk Factors

Run a risk Factors	N (%)	Hour (95% CI)	P Value	aHR (95% CI)	P Value
Year of nativity (per y)	5070 (100)	0.93 (0.91–0.94)	<0.001	0.94 (0.92–0.95)	<0.001
Gestational historic period
Very preterm (<32 weeks)	149 (2.9)	4.33 (three.3–five.67)	<0.001	6.85 (5.11–9.18)	<0.001
Moderately preterm (32–36 weeks)	619 (12.two)	two.09 (1.74–2.53)		i.87 (one.54–2.27)
Term (37–41 weeks)	4040 (79.seven)	1 (reference category)		1 (reference category)
Post‐term (≥42 weeks)	172 (3.4)	0.73 (0.43–1.21)		0.65 (0.38–1.08)
Missing	90 (1.8)
Birth weight
Low (z <−1)	1274 (59.0)	1.73 (1.48–2.03)	<0.001	1.31 (one.11–one.55)	0.001
Average (−1≤ z ≤i)	2935 (25.six)	ane (reference category)		i (reference category)
High (z >ane)	770 (15.5)	0.83 (0.65–1.06)		0.76 (0.59–0.98)
Missing	91
Extracardiac anomalies
None	4181 (82.5)	1 (reference category)	<0.001	1 (reference category)	<0.001
Structural	287 (5.7)	4.22 (3.38–v.27)		2.95 (2.34–iii.72)
Chromosomal/genetic	602 (eleven.9)	four.eleven (3.47–4.87)		three.07 (ii.57–3.67)
Maternal historic period (per y)	4795 (94.half-dozen)	i.01 (1.00–1.02)	0.04	1.01 (1.00–1.02)	0.ten
Missing	275 (5.four)
Sexual practice
Male person	2675 (52.8)	1 (reference category)	0.65	1 (reference category)	0.64
Female	2395 (47.ii)	0.96 (0.83–one.12)		1.05 (0.nine–i.22)
Deprivation
Most deprived	1690 (33.3)	1 (reference category)	0.11	ane (reference category)	0.twenty
Average deprived	1690 (33.3)	0.94 (0.78–1.12)		0.93 (0.78–one.11)
Least deprived	1689 (33.3)	0.82 (0.69–0.99)		0.84 (0.69–ane.02)
Missing	ane (0.02)
Plurality
Singleton	4918 (97.0)	1 (reference category)	0.009	1 (reference category)	0.22
Multiple	151 (3.0)	ane.61 (1.13–2.31)		0.78 (0.53–i.15)
Missing	ane (0.02)
Severity			<0.001
I (most)	163 (3.ii)	4.26 (three.45–5.25)		5.73 (4.61–seven.13)
Ii	1579 (31.1)	1 (reference category)		1 (reference category)
3 (least)	3032 (59.eight)	0.15 (0.12–0.18)		0.19 (0.15–0.23)
Unclassified	296 (5.8)	0.55 (0.xl–0.75)		0.49 (0.36–0.67)

Statistical Assay

Unadjusted and adjusted HRs were estimated using proportional hazards Royston–Parmar models, with ane knot. Parametric models such as Royston–Parmar models differ from the traditionally used semiparametric Cox model considering they straight model the baseline risk.¹⁹ This results in smoother and therefore more‐precise predictions of survival than those resulting from the Cox model. The Royston–Parmar approach in particular makes no specific distributional assumptions (as is the case for other parametric approaches such as the Weibull or exponential models), instead modeling and smoothing the baseline hazard using cubic splines to maximise model accuracy. Mean v‐year survival estimates for composite CHD were estimated using a Royston–Parmar model adjusted for nascency weight, gestational historic period, extracardiac anomalies, and yr of birth, and reported for isolated CHD (ie, no extracardiac anomalies) during the study's most contempo twelvemonth of birth (ie, 2003). Where there were 5 or more than deaths per variable, hateful survival estimates were also estimated for individual CHD subtypes. Here, Royston–Parmar models were fitted with dichotomization of gestational age (preterm [<37 weeks] versus term [≥37 weeks]) and presence of extracardiac anomalies (none versus any), attributed to low numbers. Ninety‐v pct CIs were estimated using the Delta method.

The proportional hazards assumption was checked using log‐log plots and past comparison HRs for different categorizations of survival time. Martingale residuals were plotted against continuous variables to check that the functional form was linear. P<0.05 was considered statistically pregnant and analyses were performed in Stata software (version xiv; StataCorp LP, College Station, TX).

Results

Of 5093 cases of CHD, 5070 (99.5%) had known survival status. Of these, 87.i% (95% CI, 86.2–88.0) survived to historic period five years, 86.7% (95% CI, 85.7–87.half dozen%) to age 10 years and 85.two% (95% CI, 84.1–86.3) to age 20 years. Of cases with isolated CHD, 91.2% (95% CI, 90.3–92.0) survived to age five years, 90.9% (95% CI, ninety.0–91.8) to historic period ten years, and 89.7% (95% CI, 88.five–xc.seven) to historic period twenty years.

Predictors of Survival

Twelvemonth of nascency (P<0.001), gestational age at birth (P<0.001), birth weight (P<0.001), extracardiac anomalies (P<0.001), CHD severity (P<0.001), maternal age (P=0.04), and plurality of pregnancy (P=0.009) were all crudely associated with survival (Table one). For each year increment in year of birth, the gamble of mortality decreased by vii% (60 minutes=0.93; 95% CI, 0.92–0.94). Cases born very preterm and moderately preterm were 4.33 (95% CI, three.xxx–5.67; P<0.001) and ii.09 (95% CI, 1.74–2.52; P<0.001) times more likely to result in mortality than term cases. Cases born post‐term were 27% less likely to outcome in bloodshed (60 minutes=0.73; 95% CI, 0.43–1.21), although this was not statistically significant (P=0.22). Compared with average nascency‐weight cases, low nativity weight cases were at increased adventure of mortality (HR=i.73; 95% CI, 1.48–two.03; P<0.001) and loftier birth weight cases were at decreased chance of mortality, although this did non attain statistical significance in the univariable model (HR=0.83; 95% CI, 0.65–1.06; P=0.13). For each twelvemonth increase in maternal age, the risk of mortality increased by 1% (HR=ane.01; 95% CI, one.00–1.02). The risk of mortality was 18% lower in cases resident in the region's third least deprived areas compared with the third well-nigh deprived (95% CI, 0.68–0.98; P=0.04), although the total effect of socioeconomic deprivation was not statistically pregnant (P=0.11). Cases from multiple pregnancies were ane.62 times more likely to result in mortality than singletons (95% CI, ane.13–2.32). Cases with structural and chromosomal anomalies were at quadrupled risks of mortality (Hour=4.22; 95% CI, 3.38–5.27; P<0.001 and Hour=four.xi; 95% CI, 3.47–4.87; P<0.001, respectively).

Yr of nascence (P<0.001), gestational age at nascence (P<0.001), nascency weight (P=0.001), CHD severity (P<0.001), and extracardiac anomalies (P<0.001) remained meaning in the multivariable model, although the HRs were all attenuated compared with univariable analyses (Table 1). Plurality was no longer significant (P=0.22), with the issue irresolute direction (adapted HR=0.78; 95% CI, 0.53–1.15). The small increased risk of mortality associated with increasing maternal age was similar, merely no longer statistically significant (P=0.10).

5‐Year Conditional Survival Estimates

Virtually children with CHD were born at term with boilerplate birth weight (53.7%, 54.7%, 52.1%, and 56.6%, respectively for blended CHD, severity I, II, Ii, and unclassified CHD) and only very few were both low birth weight and very preterm (0.five%, 0.4%, 0.ane%, 0.vii%, and 0.i%, respectively, for any CHD, severity I, Ii, III, and unclassified CHD).

Five‐yr survival for a child with isolated CHD, built-in at term in 2003 with average birth weight, was estimated as 96.3% (95% CI, 95.6–97.0). This dropped to 83.1% (95% CI, 78.5–87.8) for very preterm births and 93.4% (95% CI, 91.9–95.0) for moderately preterm births, only increased to 97.three% (95% CI, 95.8–98.7) for mail service‐term births. Five‐year survival for a child with isolated CHD, born at term in 2003 with low nascence weight, was estimated every bit 95.2% (95% CI, 94.2–96.3) and with loftier birth weight was estimated as 97.two% (95% CI, 96.4–98.0). Children with low nascence weight born very preterm had the worst prognosis (survival=78.8%; 95% CI, 72.8–84.7) and children with loftier birth weight built-in post‐term had the best prognosis (survival=97.9%; 95% CI, 96.8–99.i; Table 2).

Table ii. 5‐Twelvemonth Survival Estimates by CHD Subtypes

CHD Subtype	Deaths/North	Kaplan–Meier Survival Gauge for Isolated CHD Built-in 1985–2003	Provisional Survival Estimates, for Isolated CHD Born 2003a
			Term, Average Birth Weight	Preterm, Average Nascence Weight	Term, Low Nascency Weight	Preterm, Low Birth Weight	Term, High Nascency Weight	Preterm, High Nativity Weight
Severe I	108/163	34.7 (27.0–42.5)	58.8 (42.8–74.7)	38.4 (15.4–61.iv)	53.v (33.0–73.9)	32.5 (7.4–57.5)	59.7 (38.three–81.ii)	39.five (13.0–66.one)
Hypoplastic left eye	76/79	≤5.5 (ane.eight–12.4)b	···	···	···	···	···	···
Unmarried ventricle	four/eleven	77.viii (36.5–93.9)	···	···	···	···	···	···
Hypoplastic right heart	4/12	63.6 (29.vii–85.4)	···	···	···	···	···	···
Tricuspid atresia	16/34	63.0 (42.ane–78.i)	···	···	···	···	···	···
Ebstein bibelot	viii/27	lxx.8 (48.4–84.9)	···	···	···	···	···	···
Severity Ii	385/1579	81.0 (78.7–83.1)	89.2 (86.5–91.eight)	79.3 (73.v–85.0)	87.9 (84.6–91.ii)	76.9 (70.3–83.half-dozen)	90.8 (87.6–94.0)	82.2 (76.1–88.3)
Pulmonary valve atresia	28/66	61.6 (47.4–73.0)	61.2 (35.3–87.1)	63.0 (18.7–100)	38.half dozen (3.8–73.4)	forty.ix (−thirteen.iii–95.1)	67.7 (37.7–97.8)	69.3 (34.7–100)
Interrupted aortic arch	13/33	52.6 (28.7–71.9)	···	···	···	···	···	···
Common arterial trunk	35/52	36.ane (21.0–51.4)	77.2 (55.4–99.0)	54.five (14.2–94.7)	74.8 (53.6–96.0)	50.half-dozen (ix.7–91.5)	92.9 (81.4–100)	84.2 (61.1–100)
Aortic valve atresia/stenosis	30/247	89.4 (84.6–92.8)	94.6 (89.iv–99.eight)	91.v (81.half dozen–100)	93.iii (86.four–100.0)	89.half dozen (76.7–100)	97.5 (93.3–100)	96.0 (89.3–100)
Atrioventricular septal defect	90/264	76.vi (67.four–83.5)	89.two (83.0–95.4)	76.0 (61.5–xc.v)	82.four (72.1–92.7)	62.8 (41.eight–83.7)	93.6 (87.4–99.8)	85.3 (71.5–99.1)
Tetralogy of fallot	61/271	85.3 (79.5–89.half-dozen)	90.vi (84.6–96.five)	86.two (76.0–96.4)	89.three (82.two–96.four)	84.4 (72.9–95.eight)	90.iii (81.8–98.9)	85.9 (72.4–99.3)
Total anomalous pulmonary venous return	19/64	72.7 (58.ix–82.6)	···	···	···	···	···	···
Transposition of the great vessels	l/222	78.7 (72.iv–83.7)	93.1 (88.three–98.0)	74.vii (56.seven–92.vi)	92.5 (86.4–98.6)	72.5 (51.one–93.9)	94.2 (88.9–99.5)	78.ii (61.8–94.7)
Coarctation of the aorta	46/258	85.2 (79.7–89.three)	86.iii (77.4–95.1)	72.6 (52.i–93.ane)	xc.2 (82.iv–98)	79.nine (62.0–97.8)	82.8 (69.9–95.seven)	66.5 (41.6–91.5)
Double outlet right ventricle	9/22	64.three (34.3–83.iii)	···	···	···	···	···	···
Severity III	118/3032	98.8 (98.three–99.i)	99.3 (99.0–99.half-dozen)	97.8 (96.half-dozen–98.9)	99.0 (98.six–99.five)	96.nine (95.2–98.5)	99.6 (99.four–99.ix)	98.8 (97.9–99.7)
Pulmonary valve stenosis	10/428	98.4 (96.v–99.3)	···	···	···	···	···	···
Atrial septal defect	nineteen/422	98.2 (96.1–99.2)	···	···	···	···	···	···
VSD	88/2182	98.9 (98.three–99.3)	99.iii (98.9–99.7)	97.3 (95.7–98.ix)	99.2 (98.vi–99.7)	96.7 (94.vi–98.viii)	99.7 (99.four–99.nine)	98.6 (97.v–99.8)
Mitral valve anomalies	3/fourscore	97.3 (89.eight–99.3)	···	···	···	···	···	···
Unclassified severity	41/296	93.0 (88.5–95.viii)	98.1 (96.iii–99.9)	93.8 (87.viii–99.8)	95.9 (91.9–100.0)	86.ix (74.half-dozen–99.ii)	97.7 (95.3–100)	92.v (84.seven–100)
Patent ductus arteriosus	6/39	92.5 (87.9–95.4)	···	···	···	···	···	···

V‐yr survival for a child with isolated CHD, born at term in 2003 with average birth weight, was estimated as 58.eight% (95% CI, 42.8–74.vii) for a severity I CHD, 89.2% (95% CI, 86.5–91.eight) for severity II, 99.iii% (95% CI, 99–99.six) for severity III, and 98.i% (95% CI, 96.iii–99.9) for unclassified. Survival for selected CHD subtypes provisional on nativity weight and gestational age are shown in Table 2.

Discussion

Twelvemonth of birth, gestational historic period at birth, nascence weight, and extracardiac anomalies were independently associated with mortality in individuals with CHD. Most children (53.vii%) with isolated CHD were born at term with average nascency weight. An estimated 96.3% of isolated cases built-in at term in 2003, with average nativity weight, were live at age 5 years. This ranged from 58.8% for children with the about astringent CHD subtypes to 99.iii% for the to the lowest degree severe. Five‐yr survival was most optimistic (97.nine%) for children born with high birth weight post‐term and was least optimistic (78.eight%) for children born very preterm with a low nascency weight.

This report'due south primary force is the use of data from a high‐quality, population‐based register, which is notified from multiple sources to maximize ascertainment. The NorCAS is annually cross‐validated with a pediatric cardiac database at the local 3rd center. Complex cases are reviewed by pediatric pathologists and clinical geneticists, and, where relevant, diagnoses are confirmed past postmortem. NorCAS cases may be diagnosed at whatever historic period upward to 16 years (12 afterward 2001), meaning that hard and late diagnoses are included. All the same, cases born to mothers resident in the N of England that move out of the UK earlier diagnosis may non exist picked upwardly by the NorCAS. Given that the North of England is a very stable population with petty migration, this is likely to apply to a very few cases.^twenty Only 23 cases (0.5%) were untraced, virtually eliminating any potential bias from loss to follow‐upward.

Nosotros uniquely present adapted 5‐year survival estimates, overall, by CHD severity and by selected CHD subtypes. Existing studies typically study survival for all CHD combined, non accounting for modifying factors such as extracardiac anomalies or preterm nascence. We show that these factors substantially influence survival (eg, 5‐year survival for common arterial trunk falls from 77.2% in term births with average birth weight to fifty.half dozen% for preterm births with low birth weight). Adjusted survival estimates are important for wellness and social intendance planning. For prenatal counselling, they tin can provide best‐ and worst‐case scenarios, depending on the final gestational age and nascency weight. For postnatal diagnoses, they can provide parents with more‐accurate predictions based on their baby'due south birth weight and gestational age.

Although i of the largest studies to examine the influences of CHD survival, we still lacked power to assess uncommon features (eg, high nativity weight or post‐term birth) or factors with modest upshot sizes (eg, socioeconomic deprivation). Nonsignificant associations should therefore be interpreted with intendance.

Simulation studies suggest that survival analysis requires a minimum of 5 to 10 events per variable for adequate statistical power.^{21 , 22 , 23} Our adjusted survival estimates for individual subtypes and severity categories were derived from models containing iv dummy categorical variables and one continuous variable. To accurately report survival, we therefore required at least 20 deaths for each CHD subtype, which meant several subtypes could not be examined individually.

Ethnicity and parity have previously been associated with CHD mortality.^{6 , 7 , eight , 9} Data notified to the NorCAS are nerveless routinely in clinical settings, and these variables are poorly recorded. Surgical and medical interventions are also not recorded on the NorCAS, but likely affect survival. In particular, for cases of hypoplastic left heart, survival may be improved with palliative surgery, though many parents opt for comfort care.²⁴ Moreover, younger age at surgical intervention appears to better survival in children with CHD.^{25 , 26 , 27 , 28 , 29} The NorCAS does not hold data on morbidities such every bit sepsis or pulmonary hypertension, which are more than prevalent in children with CHD and increase the risk of bloodshed.^{29 , 30 , 31}

Without information on crusade of death, we cannot confirm whether a cardiac effect was the cause of death. Mortality among cases with extracardiac anomalies may outcome from the coincident bibelot, rather than the CHD. Nonetheless, for severe subtypes, the contribution of any additional anomalies is diminished by the CHD lethality. To provide the virtually widely relevant figures, nosotros presented our adapted survival estimates for isolated CHD only.

Nosotros found that CHD survival improved over fourth dimension during 1985–2003. We therefore stock-still our survival estimates to the latest year of study. Survival prospects are probable to have improved since 2003, and this should exist considered when interpreting our estimates. Assuming trends increased at the same rate until 2016, survival for cases born at term, with average birth weight and isolated CHD, would have increased form 96.3% to 98.7%. Similarly, survival for cases born very preterm with depression nativity weight would have increased from 78.viii% in 2003 to 91.9% in 2016. Survival for cases born post‐term with loftier birth weight would have increased from 97.9% in 2003 to 99.iii% in 2016.

Our Kaplan–Meier survival estimate of 87.viii% at age v years is comparable to the pooled estimate of 85% reported in our recent systematic review³ (afterward excluding Tennant et al, which used overlapping data^eighteen).

In our study, cases with extracardiac anomalies experienced ≈four‐fold increased risk of bloodshed. Knowles et al and Olsen et al similarly reported that children with extracardiac anomalies were at increased risk of mortality, but with smaller effect sizes.^{11 , 29} These discrepancies may effect from Knowles et al excluding cases with Downwards syndrome and Olsen et al having a dissimilar case mix, with just half the proportion of ventricular septal defects than our study (23% versus 43%).

Nosotros found that CHD survival improved over time, reflecting the findings of several other population‐based studies.^{5 , 8 , 11 , 32} This improvement is likely explained by many factors, including the development of several surgical interventions. For example, the Fontan functioning and the Norwood surgery were introduced in the U.k. in 1975 and 1993, respectively, to enable palliative treatment for unmarried ventricle, hypoplastic left heart, and tricuspid atresia.³³ Similarly, the arterial switch performance was introduced to the UK in 1984, replacing the atrial switch operations. Improving expertise as well every bit full general improvements in neonatal intendance (which enable survival until surgical intervention) are likely to have contributed to the ongoing improvements in survival. For case, prostaglandin was introduced to the United kingdom in 1978, helping patients to remain stable before surgical intervention.³⁴

We found that greater gestational historic period at birth was associated with improved survival. Knowles et al also reported an increased take chances of mortality in preterm compared with term cases (Hr=one.43).²⁹ The slightly smaller effect size may result from Knowles et al examining simply "serious CHD." Fixler et al similarly reported an increased risk of mortality in very preterm cases (HR=2.80) and moderately preterm cases (HR=1.69), but just included CHD subtypes with single‐ventricle physiology.^four Nosotros found that increased standardized birth weight was associated with improved survival. Wang et al and Oster et al similarly reported that increased birth weight improved survival.^{8 , 32} Cardiac operative mortality has been shown to increase with lower birth weight and lower gestational age at nascency. Furthermore, among children with CHD, low gestational age at birth poses an increased run a risk of necrotizing entercolitis.³⁵ Of course, in individuals without CHD, the risk of mortality increases as gestational age and birth weight decreases.^{36 , 37} However, our increased hazard of bloodshed associated with moderately preterm nativity in individuals with CHD (Hour=2.09) exceeds that reported by Crump et al in the general population (HR=1.lxxx for babies born 28–33 weeks and Hour=1.52 for babies born 34–36 weeks versus 37–42 weeks).³⁶

We establish some evidence of an association betwixt maternal historic period at delivery and mortality. Wang et al and Oster et al reported significantly decreased mortality in cases born to mothers respectively aged >35 years compared with xxx to 34 years (Hour=0.88)⁸ and aged ≥30 years compared with <30 years (Hour=0.77),³² respectively. Other population‐based studies have reported no significant association between CHD survival and maternal age in cases with single‐ventricle physiology^{4 , half dozen} and atrioventricular septal defect, but showed lower survival in children born to older mothers. Potentially the association with maternal age is confounded by other factors, such every bit CHD subtype, socioeconomic deprivation, and gestational age at birth, which all varied by maternal age in our data.

We found lower survival in cases from multiple pregnancies. However, later on accounting for other variables, this association was no longer significant. In our data, cases from multiple births were x times more probable to be preterm, i.5 times more likely to accept depression birth weight, and 2.5 times more probable to have structural extracardiac anomalies. Therefore, although twins on average have poorer prognoses, a term, average‐weight twin with isolated CHD should accept the same survival prospects as an equivalent singleton.

We plant poorer survival of children with CHD built-in in the virtually compared with the least deprived areas in the North of England. Miller et al did not find a significant association between socioeconomic position and atrioventricular septal defect survival in the The states; nonetheless, survival decreased linearly with decreasing level of deprivation.^ten Area‐based deprivation is a complex and multifaceted exposure, with many domains and correlates, only some of which may be related to CHD survival. Detecting such an association may crave a larger data set with greater power or more than detail on the individual features of the exposure. While the impact may be small for the individual, it may still be an important determinant at the population level.

Conclusion

Twenty‐twelvemonth survival associated with CHD was 85.2%. Year of nascency, gestational historic period at birth, standardized birth weight, and the presence of extracardiac anomalies were associated with mortality in individuals with CHD. This information is important for health and social care planning.

Acknowledgments

We thank the staff at the NorCAS and the link clinicians for their continued collaboration and support of the NorCAS.

Sources of Funding

This work was supported by a British Heart Foundation studentship (FS/12/23/29511 to Best). NorCAS is currently funded past Public Health England.

Disclosures

None.

Footnotes

*Correspondence to: Judith Rankin, PhD, Institute of Health & Society, Newcastle University, Baddiley‐Clark Edifice, Newcastle upon Tyne, England NE2 4AX, United kingdom. E‐postal service: judith. [email protected] ac.uk

References

• 1 EUROCAT . United kingdom of great britain and northern ireland built anomaly register prevalence tables. 2013. Bachelor at: http://world wide web.eurocat-network.eu/accessprevalencedata/prevalencetables. Accessed April 24, 2017.Google Scholar
• 2 Wren C, O'Sullivan JJ. Survival with congenital middle disease and need for follow up in adult life. Eye . 2001; 85:438–443.CrossrefMedlineGoogle Scholar
• 3 All-time KE, Rankin J. Long‐term survival of individuals born with congenital heart illness: a systematic review and meta‐analysis. J Am Heart Assoc . 2016; v:e002846. https://doi.org/ten.1161/jaha.115.002846.LinkGoogle Scholar
• 4 Fixler DE, Nembhard WN, Salemi JL, Ethen MK, Canfield MA. Mortality in kickoff v years in infants with functional single ventricle built-in in Texas, 1996 to 2003. Circulation . 2010; 121:644–650.LinkGoogle Scholar
• v Garne E. Congenital middle defects—occurrence, surgery and prognosis in a Danish County. Scand Cardiovasc J . 2004; 38:357–362.CrossrefMedlineGoogle Scholar
• six Idorn L, Olsen Chiliad, Jensen AS, Juul Yard, Reimers JI, Sorensen Chiliad, Johnsen SP, Sondergaard L. Univentricular hearts in Denmark 1977 to 2009: incidence and survival. Int J Cardiol . 2013; 167:1311–1316.CrossrefMedlineGoogle Scholar
• 7 Nembhard WN, Salemi JL, Ethen MK, Fixler DE, Dimaggio A, Canfield MA. Racial/ethnic disparities in risk of early babyhood bloodshed amidst children with built heart defects. Pediatrics . 2011; 127:e1128–e1138.CrossrefMedlineGoogle Scholar
• viii Wang Y, Hu J, Druschel CM, Kirby RS. Xx‐v‐yr survival of children with birth defects in New York Land: a population‐based study. Nativity Defects Res A Clin Mol Teratol . 2011; 91:995–1003.CrossrefMedlineGoogle Scholar
• 9 Wang Y, Liu Chiliad, Druschel CM, Kirby RS. Maternal race/ethnicity and survival experience of children with congenital centre disease. J Pediatr . 2013; 163:1437–1442.e1431–1432.CrossrefMedlineGoogle Scholar
• 10 Miller A, Siffel C, Lu C, Riehle‐Colarusso T, Frías JL, Correa A. Long‐term survival of infants with atrioventricular septal defects. J Pediatr . 2010; 156:994–thou.CrossrefMedlineGoogle Scholar
• xi Olsen M, Christensen TD, Pedersen Fifty, Johnsen SP, Hjortdal VE. Tardily mortality amid Danish patients with congenital heart defect. Am J Cardiol . 2010; 106:1322–1326.CrossrefMedlineGoogle Scholar
• 12 EUROCAT . EUROCAT guide 1.iv. 2014. Bachelor at: http://www.eurocat-network.eu/aboutus/datacollection/guidelinesforregistration/guide1_4. Accessed Apr 24, 2017.Google Scholar
• 13 Gardosi J, Mongelli K, Wilcox One thousand, Chang A. An adaptable fetal weight standard. Ultrasound Obstet Gynecol . 1995; half-dozen:168–174.CrossrefMedlineGoogle Scholar
• fourteen Tin W, Wariyar Great britain, Hey EN. Selection biases invalidate current low birthweight weight‐for‐gestation standards. BJOG . 1997; 104:180–185.CrossrefGoogle Scholar
• xv Noble K, Wright Thousand, Smith G, Dibben C. Measuring multiple impecuniousness at the small‐area level. Environ Programme A . 2006; 38:169.CrossrefGoogle Scholar
• 16 Department for Communities & Local Government . English indices of deprivation 2010. 2010.Google Scholar
• 17 Khoshnood B, Loane 1000, Garne Eastward, Addor MC, Arriola L, Bakker M, Barisic I, Bianca S, Boyd P, Calzolari E, Doray B, Draper E, Gatt M, Haeusler Thou, Melve KK, Latos‐Bielenska A, McDonnell B, Mullaney C, Nelen V, O'Mahony Chiliad, Pierini A, Queisser‐Luft A, Randrianaivo H, Rankin J, Rissmann A, Salvador J, Tucker D, Verellen‐Dumoulin C, Wellesley D, Zymak‐Zakutnya N, Dolk H. Recent Decrease in the Prevalence of Built Heart Defects in Europe. J Pediatr. 2012; 162:108–113.CrossrefMedlineGoogle Scholar
• 18 Tennant PW, Pearce MS, Bythell M, Rankin J. 20‐twelvemonth survival of children born with congenital anomalies: a population‐based written report. Lancet . 2010; 375:649–656.CrossrefMedlineGoogle Scholar
• 19 Royston P, Lambert P. Flexible Paramteric Survival Analyses Using Stata: Across the Cox Model . Texas: Stata Press; 2011.Google Scholar
• 20 Hodgson S, Shirley M, Bythell Grand, Rankin J. Residential mobility during pregnancy in the due north of England. BMC Pregnancy Childbirth . 2009; 9:52.CrossrefMedlineGoogle Scholar
• 21 Concato J, Peduzzi P, Holford TR, Feinstein AR. Importance of events per independent variable in proportional hazards assay I. Background, goals, and full general strategy. J Clin Epidemiol . 1995; 48:1495–1501.CrossrefMedlineGoogle Scholar
• 22 Peduzzi P, Concato J, Feinstein AR, Holford TR. Importance of events per independent variable in proportional hazards regression analysis. 2. Accurateness and precision of regression estimates. J Clin Epidemiol . 1995; 48:1503–1510.CrossrefMedlineGoogle Scholar
• 23 Vittinghoff E, McCulloch CE. Relaxing the rule of 10 events per variable in logistic and Cox regression. Am J Epidemiol . 2007; 165:710–718.CrossrefMedlineGoogle Scholar
• 24 Fruitman DS. Hypoplastic left heart syndrome: prognosis and direction options. Paediatr Child Health . 2000; 5:219.CrossrefMedlineGoogle Scholar
• 25 Mahle WT, Spray TL, Wernovsky Thousand, Gaynor JW, Clark BJ. Survival after reconstructive surgery for hypoplastic left middle syndrome a 15‐twelvemonth experience from a single institution. Circulation . 2000; 102:3‐136–Three‐141.LinkGoogle Scholar
• 26 Murphy JG, Gersh BJ, McGoon MD, Mair DD, Porter CJ, Ilstrup DM, McGoon DC, Puga FJ, Kirklin JW, Danielson GK. Long‐term upshot after surgical repair of isolated atrial septal defect: follow‐up at 27 to 32 years. N Engl J Med . 1990; 323:1645–1650.CrossrefMedlineGoogle Scholar
• 27 Pigula FA, Khalil PN, Mayer JE, Pedro J, Jonas RA. Repair of tetralogy of fallot in neonates and young infants. Circulation . 1999; 100:Ii‐157–Ii‐161.LinkGoogle Scholar
• 28 Boening A, Scheewe J, Heine Yard, Hedderich J, Regensburger D, Kramer HH, Cremer J. Long‐term results after surgical correction of atrioventricular septal defects. Eur J Cardiothorac Surg . 2002; 22:167–173.CrossrefMedlineGoogle Scholar
• 29 Knowles RL, Bull C, Wren C, Wade A, Goldstein H, Dezateux C. Modelling survival and mortality risk to 15 years of historic period for a national cohort of children with serious congenital heart defects diagnosed in infancy. PLoS One . 2014; ix:e106806.CrossrefMedlineGoogle Scholar
• 30 Ascher SB, Smith Lead, Clark RH, Cohen‐Wolkowiez Chiliad, Li JS, Watt K, Benjamin DK. Sepsis in immature infants with congenital center affliction. Early Hum Dev . 2012; 88:S92–S97.CrossrefMedlineGoogle Scholar
• 31 van Loon RLE, Roofthooft MTR, Hillege HL, ten Harkel ADJ, van Osch‐Gevers M, Delhaas T, Kapusta Fifty, Strengers JLM, Rammeloo L, Clur Southward‐AB, Mulder BJM, Berger RMF. Pediatric pulmonary hypertension in the Netherlands Clinical Perspective. Epidemiology and characterization during the catamenia 1991 to 2005. Circulation . 2011; 124:1755–1764.LinkGoogle Scholar
• 32 Oster ME, Lee KA, Honein MA, Riehle‐Colarusso T, Shin M, Correa A. Temporal trends in survival amidst infants with disquisitional congenital middle defects. Pediatrics . 2013; 131:e1502–e1508.CrossrefMedlineGoogle Scholar
• 33 Knowles RL, Bull C, Wren C, Dezateux C. Mortality with congenital heart defects in England and Wales, 1959–2009: exploring technological change through menstruation and nascence cohort analysis. Arch Dis Child . 2012; 97:861–865.CrossrefMedlineGoogle Scholar
• 34 Olley PM, Coceani F, Bodach E. Due east‐type prostaglandins: a new emergency therapy for certain cyanotic congenital heart malformations. Circulation . 1976; 53:728–731.LinkGoogle Scholar
• 35 McElhinney DB, Hedrick HL, Bush DM, Pereira GR, Stafford PW, Gaynor JW, Spray TL, Wernovsky Yard. Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes. Pediatrics . 2000; 106:1080–1087.CrossrefMedlineGoogle Scholar
• 36 Crump C, Sundquist 1000, Sundquist J, Winkleby MA. Gestational historic period at birth and mortality in immature adulthood. JAMA . 2011; 306:1233–1240.CrossrefMedlineGoogle Scholar
• 37 Risnes KR, Vatten LJ, Baker JL, Jameson Chiliad, Sovio U, Kajantie E, Osler 1000, Morley R, Jokela M, Painter RC, Sundh V, Jacobsen GW, Eriksson JG, Sorensen TI, Bracken MB. Birthweight and mortality in adulthood: a systematic review and meta‐assay. Int J Epidemiol . 2011; 40:647–661.CrossrefMedlineGoogle Scholar

davistherveld.blogspot.com

Source: https://www.ahajournals.org/doi/10.1161/JAHA.116.005213

Share this post