The impact of prematurity and associated comorbidities on clinical outcomes in neonates with congenital heart disease

Prematurity is a common risk factor in children, affecting approximately 10% of live births, globally. It is more common in children with critical congenital heart disease (CCHD) and carries important implications in this group of patients. While outcomes have been improving over the years, even late preterm birth is associated with worse outcomes in children born with critical congenital heart disease compared to those without. Infants with both prematurity and CCHD are at particularly high risk for important comorbidities, including: necrotizing enterocolitis, intraventricular hemorrhage, white matter injury, neurodevelopmental anomalies and retinopathy of prematurity. Lesion-specific intensive care management of these infants, interventional and peri-operative management specifically tailored to their needs, and multidisciplinary care all have the potential to improve outcomes in this challenging group.     

Feeding abilities in neonates with congenital heart disease: a retrospective study

OBJECTIVE: An important area concerning morbidity among infants with congenital heart defects (CHD) is related to feeding problems. Our objectives were to characterize the evolution of feeding milestones related to transition to per oral feeding among infants with CHD, and to identify associated variables impacting the feeding abilities. Specifically, we differentiated the feeding characteristics in neonates with acyanotic vs cyanotic CHD. STUDY DESIGN: Feeding progress was tracked during the first hospitalization in a retrospective chart review study involving 76 infants (29 acyanotic, 47 cyanotic CHD). The ages at which the following milestones attained were recorded: first feeds, maximum gavage feeds, first nipple feeds and maximum nipple feeds, in addition to the length of hospital stay. Effects of perinatal factors, duration of respiratory support, vasopressor and narcotic use and use of cardiopulmonary bypass on the feeding milestones were also evaluated. ANOVA, t-test, and stepwise linear regression analysis were applied as appropriate. Data stated as mean+/-s.e.m., or %; P<0.05 was considered significant. RESULT: Prenatal and birth characteristics were similar (P=NS) between the neonates with acyanotic and cyanotic CHD. Cyanotic CHD required three times prolonged use of ventilation, narcotics and vasopressor use (all P<0.05, compared to the acyanotic group). In the acyanotic group, prolonged respiratory support correlated linearly with time to attain maximal gavage feeds and nippling (both, R2=0.8). In the cyanotic group, delayed initiation of gavage feeds and prolonged respiratory support both correlated linearly with time to attain maximal gavage feeds and nippling (both, R2=0.8). Age at first gavage feed correlated with maximum gavage feeds among neonates with cyanotic CHD, and first nipple feed correlated with maximum nipple feeds among all groups (P<0.01). Use of cardiopulmonary bypass in cyanotic CHD delayed the feeding milestones and prolonged the length of stay (both, P<0.05 vs non-bypass group); similar findings were not seen in the acyanotic group. CONCLUSION: In contrast to neonates with acyanotic CHD, cyanotic CHD group had significant delays with (a) feeding readiness, (b) successful gastric feeding, (c) oromotor readiness and (d) successful oromotor skills. Co-morbid factors that may directly influence the delay in feeding milestones include the (a) duration of respiratory support and (b) use of cardiopulmonary bypass. Delays in achieving maximum gavage and maximum nippling may suggest foregut dysmotility and oropharyngeal dysphagia.     

Long-term and developmental outcomes of children with complex congenital heart disease

As short-term survival of complex congenital heart disease continues to improve dramatically with advances in medical and surgical treatment, further efforts must be made to understand the long-term outcomes of our efforts. As survival continues to improve, cardiovascular morbidity and, equally importantly, neurodevelopmental and social outcomes must be a continual focus in our treatment of these complex patients. Further study of these effects is underway, and more is certainly warranted. Understanding should lead to modification of current techniques and management strategies, all with the ultimate goal of improving our patients' quality of life.     

Characteristics of neonates whose mothers have congenital and acquired heart disease

It was made a study of the body dimensions, Apgar evaluation, and frequency of innate body defects in 141 new-borns by mothers with innate and acquired heart valvular defects. It was found that the average values of body weight, height, and the evaluation with Apgar of the new-borns by mothers with heart defects do not deviate from the average values of the same indices of new-borns by healthy mothers. Despite that 90% of the young mothers with heart defects are with light to moderate degree of the heart defect, and with I or II functionality class, the percent of their new-borns born prematurely is big--31.9, compared to 6.6 for new-borns by healthy mothers. In 2.8% of the new-borns by mothers with heart defects, hypotrophy is observed, and in 3.5%--clinical criteria for immaturity. Perinatal death rate is 0.7% and zero for the control group of new-borns--ones born by healthy women. Six cases (6.7%) of new-borns by mothers with heart defects had innate body defects, 4 (4.5%) of which were innate heart defects. The frequency and the kind of the congenital defects in new-borns by mothers with heart defects is discussed.     

Neurologic events in neonates treated surgically for congenital heart disease.

OBJECTIVE: The incidence of acute neurologic events prior to discharge in neonates with congenital heart disease (CHD) was determined and peri-operative characteristics predictive of a neurologic event were identified. STUDY DESIGN: A retrospective chart review over 1 year was conducted of infants <1 month of age with a diagnosis of CHD. Outcomes were measured by the occurrence of an acute neurologic event defined as electroencephalogram (EEG)-proven seizure activity, significant hypertonia or hypotonia, or choreoathetosis prior to hospital discharge. Stepwise logistic regression identified variables most likely to be associated with an acute neurologic event. RESULTS: Surgical intervention occurred in 95 infants who were admitted with a diagnosis of CHD. The survival rate was 92%. Of the survivors, 16 (17%) had an acute neurologic event, with 19% of events occurring preoperatively. Factors associated with neurologic events included an elevated nucleated red blood cell (NRBC) count, an abnormal preoperative brain imaging study, and a 5-min Apgar score <7 (P<0.05). CONCLUSIONS: Neonates with CHD have a significant risk of neurologic events. Preoperative brain imaging, the 5-min Apgar score, and initial serum NRBC counts may identify infants at highest risk for central nervous system injury.     

Discharging neonates with congenital heart disease after cardiac surgery: a practical approach

Discharging neonates to home after cardiac surgery takes time, effective communication, and a commitment to continuity of care. The efforts of all members of a multidisciplinary team are necessary and valuable to ensure success. The discharge process involves many steps beginning at the time of admission and continuing past the actual discharge date. Discharge planning is an evolving process rather than a single event.     

Pregnancy outcomes and relative risk factors among Chinese women with congenital heart disease

ObjectiveTo summarize pregnancy outcomes among a cohort of Chinese women with congenital heart disease (CHD) and to identify predictive risk factors for maternal, fetal, and neonatal complications.MethodsA retrospective analysis of 529 women with CHD who gave birth at the Shanghai Obstetrical Cardiology Intensive Care Center, Shanghai, China, between January 1, 1993, and September 30, 2010. Maternal, fetal, and neonatal complications were evaluated for these women.ResultsMaternal cardiac complications were reported in 33 (6.2%) of the women, whereas fetal and neonatal complications were reported in 145 (27.4%). The factors found to be significantly predictive for maternal cardiac complications were cardiac events before pregnancy (odds ratio [OR] 36.811), New York Heart Association functional class greater than II (OR 27.702), oxygen saturation below 90% (OR 7.506), and left ventricular obstruction (aorta stenosis) (OR 2.408). Factors significantly predictive for fetal and neonatal complications were New York Heart Association functional class greater than II (OR 20.12), oxygen saturation below 90% (OR 3.78), and pulmonary arterial hypertension (systolic pressure ≥ 50 mm Hg) (OR 3.42).ConclusionIdentification of maternal, fetal, and neonatal complications among women with CHD may guide medical intervention and therefore reduce pregnancy-associated risk for these patients.     

Impact of perinatal management on neurodevelopmental outcomes in congenital heart disease

With advancements in cardiopulmonary bypass technique and perioperative care, there has been a progressive decline in mortality associated with neonatal surgical correction of congenital heart disease (CHD). Thus, there is now increased focus on improving neurodevelopmental outcomes in CHD survivors. While the cause of these neurodevelopmental impairments is multifactorial, there is increasing evidence that structural and functional cerebral abnormalities are present before cardiac corrective repair. This suggests that in addition to patient specific risk factors, underlying cardiac physiology and clinical hemodynamics are critical to brain health and development. Prenatal diagnosis of CHD and subsequent optimization of perinatal care may therefore be important modifiable factors for long-term neurodevelopmental outcome.This article reviews the impact that prenatal diagnosis of CHD has on perinatal care and the preoperative clinical status of a neonate, as well as the potential influence this may have on lessening the degree of cerebral injury and long-term neurodevelopmental impairments.     

妊娠合并先天性心脏病伴肺动脉高压患者的围产结局分析

目的 探讨妊娠合并先天性心脏病（先心病）伴肺动脉高压患者的围产结局。方法 收集1995年4月至2007年5月温州医学院附属第一医院产科收治的45例妊娠合并先心病伴肺动脉高压患者的临床资料,根据肺动脉压力情况分为轻度组29例[30～49mmHg（1mmHg=0．133kPa）],中度组8例（50～79mmHg）,重度组8例（≥80mmHg）,分析各组先心病的种类、心功能级别、终止妊娠孕周和方式、先心病术后妊娠的安全性以及围产结局。结果 （1）先心病种类中以房间隔缺损、室间隔缺损为主,发生率为58％（26／45）。孕前行心脏矫正手术13例（29％,13／45）,以轻度肺动脉高压、心功能Ⅰ～Ⅱ级为主。（2）轻度组心功能Ⅰ～Ⅱ级者28例,中度组心功能Ⅰ～Ⅱ级者6例,重度组心功能Ⅲ～Ⅳ级者7例。（3）轻度组足月分娩27例、难免流产1例、早产1例,新生儿平均出生体重为（3153±399）g;中度组足月分娩5例,早产3例;重度组足月分娩5例,医源性终止妊娠1例,早产2例;中、重度组新生儿平均出生体重明显低于轻度组,差异有统计学意义（P〈0．05）,同时中、重度组胎儿结局不良。（4）妊娠合并先心病伴肺动脉高压患者的分娩方式以剖宫产分娩为主,占78％（35／45）,其中轻度组剖宫产分娩22例（22／29）,中度组6例（6／8）,重度组7例（7／8）。（5）45例患者中有11例于孕前或产后出现心功能衰竭早期表现或心功能衰竭,均分布在中、重度组,且心功能均为Ⅲ～Ⅳ级。其中2例死亡。结论 妊娠合并先心病伴肺动脉高压患者的肺动脉压力越高,围产结局越差;孕前行心脏矫正手术后的先心病患者以轻度肺动脉高压为主,且围产结局良好;以剖宫产术终止妊娠是比较安全的分娩方式。     

Perinatal outcomes of congenital heart disease after emergent neonatal cardiac procedures

Purpose: We compared outcome of neonates with prenatal and post-natal diagnosis of congenital heart disease presenting in our paediatric heart centre between March 2005 and May 2015 who underwent an emergent intervention within 48?h post-partum.

Materials and methods: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized foetal echocardiography, diagnosis was made post-natally. In 98/111 (88.2%), 30-day outcome was known.

Results: Regarding the entire cohort, 30-day survival did not differ significantly in prenatal and post-natal diagnosis group (71.2 vs. 72.9%; p?>?.1). Infants with prenatal diagnosis were more likely to be born by caesarean section (59.6% vs. 33.9%, p?=?.01). Those with post-natal diagnosis had a higher need for intubation (32.7% vs. 52.5%; p?p?=?.075).

Conclusion: For newborns who require emergent neonatal cardiac procedures, our results point towards a lower death rate after prenatal diagnosis of HLH/HLHC.     

Delivery room and early postnatal management of neonates who have prenatally diagnosed congenital heart disease

Advances in fetal echocardiography are providing highly accurate diagnoses of congenital heart disease prior to delivery, making it possible to plan the delivery-room management of these newborns. Knowledge of the expected transitional circulation occurring with birth and the pathophysiologic implications of congenital heart disease increases the likelihood of providing efficient and effective therapies. The majority of neonates who have congenital heart disease will not require delivery room resuscitation in excess of routine care; however, a small number of prenatally diagnosed cardiac lesions are more likely to require urgent postnatal intervention immediately following delivery. These cardiac lesions include transposition of the great arteries with intact ventricular septum and restrictive atrial septum, hypoplastic left heart syndrome with intact atrial septum, obstructed total anomalous pulmonary venous return, and complete congenital heart block. Prenatal diagnosis allows for coordination of care surrounding delivery and during the early postnatal hours.     

Pregnancy outcomes in women with heart disease

Aim

To evaluate the maternal and fetal outcome of pregnancies complicated by cardiac disease in a developing country.

Methods

A retrospective analysis was carried out of 144 pregnancies in women with cardiac disease who delivered in our unit between 1997 and 2006. Perinatal and maternal outcomes were interpreted according to the type of the heart disease and status of the patient according to the New York Heart Association (NYHA) classification.

Results

The rates of rheumatic and congenital heart disease were 87.5 and 12.5%, respectively. The distribution of the patients according to the NYHA functional classification were 55.6, 36.1 and 8.3% for NYHA classes I, II and III–IV, respectively. There was no maternal mortality. Maternal morbidity was observed in 16 (11.1%) cases. Six perinatal mortalities (4.2%) occurred in this series. There were no significant difference in birth weight, gestational age at delivery and perinatal morbidity between the NYHA stage I–II and stage III–IV groups (P > 0.05), whereas maternal morbidity and cesarean delivery rates were significantly higher in the NYHA stage III–IV group (P < 0.001).

Conclusion

Rheumatic heart disease with pregnancy is still predominant in Turkey. Most of the patients were in a good functional group. Maternal morbidity strongly correlates with maternal cardiac classification.     

Pregnancy and delivery in women with congenital heart disease

OBJECTIVES: Perinatal care of women with congenital heart disease is very important for obstetrician. DESIGN: The purpose of this study was to analyse the course of pregnancy, delivery and after birth period of women with congenital heart disease. MATERIAL AND METHODS: A group of 35 pregnancies and deliveries in patients with congenital heart disease cured in 1998-2003 in the Department of Obstetrics and of Medical University in Gdansk were analysed. The control group was a total of 1657 deliveries in the Department of Obstetrics in the 1998. For statistical analysis of the results the T-Student test was used with the significance level p = 0.05. RESULTS: Time of pregnancy, birth weight and length were statistically different to the control group. CONCLUSIONS: We found no cardiological complications during pregnancy in patients with congenital heart disease at I and II stage NYHA. Time of pregnancy, birth weight and length were statistically different to the control group.     

Total cavopulmonary connection for surgical treatment of complex congenital heart disease.

Diversion of vena caval flow directly into the pulmonary circulation--total cavopulmonary connection--has been tried at our hospital for two years as an alternative to the modified Fontan procedure for surgical treatment of complex congenital heart disease other than tricuspid atresia in 26 cases with six operative mortalities. The causes of death were low cardiac output in four, uncontrollable paroxysmal supraventricular tachycardia in one and airway obstruction in one. Except for one late death which occurred three months after surgery due to sepsis, the 19 patients who survived the operation were followed up for four to 27 months (mean 15 months). All of them improved clinically (NYHA Class 1). Graded bicycle exercise tests were performed in five of them at three to 12 months after surgery, and their exercise tolerance was comparable to that of patients with an atriopulmonary connection. In conclusion, total cavopulmonary connection is an acceptable alternative to the modified Fontan operation for surgical treatment of complex congenital heart disease, although longer follow-up is necessary.     

ECMO and congenital heart disease

Although initially designed for respiratory failure, venoarterial extracorporeal membrane oxygenation (VA ECMO) has become a mainstay of therapy in the treatment of patients with congenital heart disease, providing preoperative and postoperative support for infants with temporary impairment of myocardial function. Postoperative support for pulmonary hypertension has allowed dramatic improvements in lesions such as total anomalous pulmonary venous connection. VA ECMO has also proven to be an acceptable bridge to cardiac transplantation in patients too small for currently available ventricular assist devices. Intraoperative elective use of ECMO has also provided a surgical environment with some specific advantages over conventional cardiopulmonary bypass for lesions such as long segment tracheal stenosis. The results of "rescue ECMO" or extracorporeal cardiopulmonary resuscitation (ECPR) has shown that many children with congenital heart disease are very salvageable despite sudden decompensation and arrest. These techniques of VA ECMO have evolved and improved over the last two decades, allowing expanded application of this life saving support.     

Obstetric and perinatal outcomes in pregnancies with isolated foetal congenital heart abnormalities

Objective: To determine obstetric, intrapartum, and perinatal outcomes for pregnancies with isolated foetal congenital heart defects (CHDs).

Methods: This was a retrospective cohort study of women that delivered an infant with an isolated major CHD between January 2010 and April 2017 at a major Australian perinatal centre. The study cohort was compared with a cohort of women with infants without CHD. Cardiac abnormalities were broadly subdivided into the following five categories using the International Classification of Diseases Tenth Revision (ICD-10) as a guide – transposition of the great arteries (TGA), septal defects, right heart lesions (RHL), left heart lesions (LHL), and “other”. Demographic characteristics and obstetric, intrapartum, and perinatal outcomes were compared between the two cohorts.

Results: The final study cohort comprised of 342 infants with isolated CHD and 68,911 controls. Of the infants with CHD, 20.4% (70/342) had transposition of the great vessels, 23% (79/342) had septal lesions, 14.6% (50/342) had right sided lesions, 23.3% (80/342) left sided, and 18.4% (63/342) categorised as “other”. Women with foetal CHD had a higher BMI and had higher rates of cardiac disease, diabetes mellitus, and hypertension, be smokers and consume alcohol compared to controls. The CHD cohort had lower odds of spontaneous vaginal delivery (SVD) (OR 0.73, 95%CI 0.58–0.90) and higher odds of caesarean for nonreassuring foetal status (aOR 1.65, 95%CI 1.07–2.55), birth weight <5th (aOR 3.44, 95%CI 2.38–4.98) and <10th (aOR 2.49, 95%CI 1.82–3.40) centiles, neonatal intensive care unit (NICU) admission (aOR 109.14, 95%CI 74.44–160.02), severe respiratory distress (aOR 2.90, 95%CI 2.33–3.76), 5?minutes Apgar score <7 (aOR 2.48, 95%CI 1.46–4.20), severe acidosis (aOR 1.80, 95%CI 1.14–2.85), stillbirth (aOR 4.09, 95%CI 1.62–10.33), neonatal death (aOR 24.30, 95%CI 13.24–44.61), and overall perinatal death (aOR 13.42, 95%CI 8.08–22.30). Infants with TGA had the lowest overall risk of complications whilst infants with RHL, LHL, and “others” had the highest risk of adverse outcomes, particularly death.

Conclusion: Infants with CHD have overall worse obstetric and perinatal outcomes compared with controls. Infants with TGA have the best perinatal outcomes of all the CHD subcategories.     

Prostaglandin induced cortical hyperostosis in neonates with cyanotic heart disease

OBJECTIVE: To study the side effects of prolonged prostaglandin therapy especially hyperostosis and other musculoskeletal changes. METHODS: Case files of the neonates, with cyanotic heart disease, who had received prostaglandin infusion from early days of life, were reviewed. Patients with periosteal changes were identified. Their radiographs, serum alkaline phosphatase activity, duration and dose of prostaglandin and other side effects related to the prostaglandin were studied. RESULTS: Ten patients developed hyperostosis, who had received PGE1 infusion for a period of 9 to 195 days. Two babies developed coarse facial features, hypertrichosis, and edema of extremities and digital swelling. Serum alkaline phosphatase activity was significantly raised in the patients, with hyperostosis. Besides long bones, ribs and scapulae, the clavicles were also involved. The involvement of clavicles has not been previously reported. CONCLUSION: Hyperostosis is a common side effect of prolonged prostaglandin therapy. Abnormal facial features, hypertrichosis and coarse skin are additional adverse effects of long term therapy. Serum alkaline phosphatase activity can be used as a marker of the hyperostosis. In addition to other bones clavicles can also be involved. The above effects seem to be both dose and duration dependent.