Association between patent ductus arteriosus flow and home oxygen therapy in extremely preterm infants.

BACKGROUND: Central blood flow measurements include the estimation of right and left ventricular output (RVO, LVO), superior vena cava (SVC) flow, and calculated patent ductus arteriosus (PDA) flow. We aimed to provide an overview of the maturation patterns of these values and the relationship between PDA flow and the need for home oxygen therapy. METHODS: This prospective single-center study was conducted in infants born at <26 weeks of gestation. We performed echocardiographic measurements five times during their life (from the 4th post-natal day to the 36th postmenstrual week). RESULTS: Sixty patients with a mean birth weight of 680 (590, 760) g were included. Postnatal development of LVO and PDA flow peaked at the end of the second postnatal week (427 and 66 mL/kg/min, respectively). The RVO increased between days 4 and 7-8. The SVCF was most stable. The development curves of PDA flow differed between the groups with (n = 28; 47%) and without home oxygen therapy. CONCLUSION: We present the central blood flow values and their postnatal development in infants <26 weeks of gestation. This study demonstrates the association between PDA flow and the future need for home oxygen therapy. IMPACT: This study enriches our knowledge of the long-term development of central blood flow parameters and derived patent ductus arteriosus (PDA) flow in extremely preterm infants (<26 weeks). While pulmonary resistance decreased, PDA flow continued to increase from day 4 to the end of the second week of life. Similarly, left ventricular output increased as a marker of preload. The superior vena cava flow remained stable. The observed association between PDA flow and an unfavorable respiratory outcome is important for future studies focusing on the prevention of chronic lung disease.

Hemodynamics During Development and Postnatal Life.

A well-developed heart is essential for embryonic survival. There are constant interactions between cardiac tissue motion and blood flow, which determine the heart shape itself. Hemodynamic forces are a powerful stimulus for cardiac growth and differentiation. Therefore, it is particularly interesting to investigate how the blood flows through the heart and how hemodynamics is linked to a particular species and its development, including human. The appropriate patterns and magnitude of hemodynamic stresses are necessary for the proper formation of cardiac structures, and hemodynamic perturbations have been found to cause malformations via identifiable mechanobiological molecular pathways. There are significant differences in cardiac hemodynamics among vertebrate species, which go hand in hand with the presence of specific anatomical structures. However, strong similarities during development suggest a common pattern for cardiac hemodynamics in human adults. In the human fetal heart, hemodynamic abnormalities during gestation are known to progress to congenital heart malformations by birth. In this chapter, we discuss the current state of the knowledge of the prenatal cardiac hemodynamics, as discovered through small and large animal models, as well as from clinical investigations, with parallels gathered from the poikilotherm vertebrates that emulate some hemodynamically significant human congenital heart diseases.

The aorto-left ventricular tunnel from a fetal perspective: Original case series and literature review.

INTRODUCTION: Aorto-left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. METHODS: All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow-up. Additionally, a systematic search of the literature was performed. RESULTS: Twenty fetuses with ALVT were identified in 10 participating centers (2001-2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac-thorax ratio (95%), left ventricular end-diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19-24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live-born (median GA 38 + 4, range 37-40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1-17). The literature reported 22 ALVT fetuses with similar outcome. CONCLUSIONS: In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome.

Left ventricular apical pacing in children: feasibility and long-term effect on ventricular function.

AIMS: Left ventricular apical pacing (LVAP) has been reported to preserve left ventricular (LV) function in chronically paced children with complete atrioventricular block (CAVB). We sought to evaluate long-term feasibility of LVAP and the effect on LV mechanics and exercise capacity as compared to normal controls. METHODS AND RESULTS: Thirty-six consecutive paediatric patients with CAVB and LVAP in the absence (N = 22) or presence of repaired structural heart disease (N = 14, systemic LV in all) and 25 age-matched normal controls were cross-sectionally studied after a median of 3.9 (interquartile range 2.1-6.8) years of pacing using echocardiography and exercise stress testing. Pacemaker implantation was uneventful and there was no death. Probability of the absence of pacemaker-related surgical revision (elective generator replacement excluded) was 89.0% at 5 years after implantation. Left ventricular apical pacing patients had lower maximum oxygen uptake (P = 0.009), no septal to lateral but significant apical to basal LV mechanical delay (P < 0.001) which correlated with decreased LV contraction efficiency (P = 0.001). Left ventricular ejection fraction and global longitudinal LV strain were, however, not different from controls. Results were similar in both the presence and absence of structural heart disease. CONCLUSION: Left ventricular apical pacing is technically feasible with a low reintervention rate. Mechanical synchrony between LV septum and free wall is maintained at the price of an apical to basal mechanical delay associated with LV contraction inefficiency as compared to healthy controls. Global LV systolic function is, however, not negatively affected by LVAP.

Is left bundle branch block pattern on the ECG caused by variable ventricular activation sequence?

BACKGROUND: The presence and extent of ventricular dyssynchrony are currently assessed from the QRS complex morphology and width. However, similar electrocardiography (ECG) pattern may be caused by variable ventricular activation sequence. This may then contribute to interindividually different response to cardiac resynchronization therapy (CRT). METHODS: Electroanatomical mapping and magnetic resonance imaging scan were performed in 11 patients with left bundle branch block (LBBB, QRS 170 ± 14 ms) and heart failure of ischemic (coronary artery disease (CAD), n = 2) and nonischemic (dilated cardiomyopathy (DCM), n = 9) etiology. Ventricular activation sequence was studied during LBBB and final CRT programming. Presence and extent of scarring were analyzed in the 17-segment left-ventricular (LV) model. RESULTS: Regardless of etiology, presence of typical LBBB was associated with diffuse prolongation of impulse conduction with right-to-left activation sequence. Basal lateral wall was constant site of late activation. This activation pattern was present in "true LBBB," but also in LBBB-like pattern (persistent S wave in V5-6) and left axis deviation. Activation started in right vetricular (RV) apex in patients with left axis deviation at RV free wall in normal axis. Individuals with CAD and DCM patient displayed focal scar. Despite that they exhibited typical LBBB and activation sequence mirrored findings in other LBBB individuals. Reverse remodeling (∆LVESV > 15% after 6 months) was evident in 10 patients. CONCLUSIONS: Both typical LBBB and LBBB-like pattern might be associated with constant activation sequence regardless of etiology and scar localization. Activation initiation in RV apex, not LV activation sequence can be surrogate for left axis deviation. CRT caused inter- and intraventricular LV resynchronization without significantly changed RV activation sequence and duration.

Does First-Trimester Screening Modify the Natural History of Congenital Heart Disease? Analysis of Outcome of Regional Cardiac Screening at 2 Different Time Periods.

BACKGROUND: The study analyzed the impact of first-trimester screening on the spectrum of congenital heart defects (CHDs) later in pregnancy and on the outcome of fetuses and children born alive with a CHD. METHODS: The spectrum of CHDs, associated comorbidities, and outcome of fetuses, either diagnosed with a CHD in the first trimester (Group I, 127 fetuses) or only in the second-trimester screening (Group II, 344 fetuses), were analyzed retrospectively between 2007 and 2013. Second-trimester fetuses diagnosed with a CHD between 2007 and 2013 were also compared with Group III (532 fetuses diagnosed with a CHD in the second trimester from 1996 to 2001, the period before first-trimester screening was introduced). RESULTS: The spectrum of CHDs diagnosed in the first and second trimesters in the same time period differed significantly, with a greater number of comorbidities (P<0.0001), CHDs with univentricular outcome (P<0.0001), intrauterine deaths (P=0.01), and terminations of pregnancy (P<0.0001) in Group I compared with Group II. In Group III, significantly more cases of CHDs with univentricular outcome (P<0.0001), intrauterine demise (P=0.036), and early termination (P<0.0001) were identified compared with fetuses diagnosed with CHDs in the second trimester between 2007 and 2013. The spectrum of CHDs seen in the second-trimester groups differed after first-trimester screening was implemented. CONCLUSIONS: First-trimester screening had a significant impact on the spectrum of CHDs and the outcomes of pregnancies with CHDs diagnosed in the second trimester. Early detection of severe forms of CHDs and significant comorbidities resulted in an increased pregnancy termination rate in the first trimester.

Long-term cognitive and cardiac outcomes after prenatal exposure to chemotherapy in children aged 18 months or older: an observational study.

BACKGROUND: Chemotherapy for the treatment of maternal cancers during pregnancy has become more acceptable in the past decade; however, the effect of prenatal exposure to chemotherapy on cardiac and neurodevelopmental outcomes of the offspring is still uncertain. We aimed to record the general health, cardiac function, and neurodevelopmental outcomes of children who were prenatally exposed to chemotherapy. METHODS: We did an interim analysis of a multicentre observational cohort study assessing children who were prenatally exposed to maternal cancer staging and treatment, including chemotherapy. We assessed children at birth, at age 18 months, and at age 5-6, 8-9, 11-12, 14-15, or 18 years. We did clinical neurological examinations, tests of the general level of cognitive functioning (Bayley or intelligence quotient [IQ] test), electrocardiography and echocardiography, and administered a questionnaire on general health and development. From age 5 years, we also did audiometry, the Auditory Verbal Learning Test, and subtasks of the Children's Memory Scale, and the Test of Everyday Attention for Children, and we also completed the Child Behavior Checklist. This study is registered with ClinicalTrials.gov, number NCT00330447. FINDINGS: 236 cycles of chemotherapy were administered in 68 pregnancies. We assessed 70 children, born at a median gestational age of 35·7 weeks (range 28·3-41·0; IQR 3·3; 47 women at <37 weeks), with a median follow-up period of 22·3 months (range 16·8-211·6; IQR 54·9). Although neurocognitive outcomes were within normal ranges, cognitive development scores were lower for children who were born preterm than for those born at full term. When controlling for age, sex, and country, the score for IQ increased by an average 11·6 points (95% CI 6·0-17·1) for each additional month of gestation (p<0·0001). Our measurements of the children's behaviour, general health, hearing, and growth corresponded with those of the general population. Cardiac dimensions and functions were within normal ranges. We identified a severe neurodevelopmental delay in both members of one twin pregnancy. INTERPRETATION: Fetal exposure to chemotherapy was not associated with increased CNS, cardiac or auditory morbidity, or with impairments to general health and growth compared with the general population. However, subtle changes in cardiac and neurocognitive measurements emphasise the need for longer follow-up. Prematurity was common and was associated with impaired cognitive development. Therefore, iatrogenic preterm delivery should be avoided when possible. FUNDING: Research Foundation-Flanders; Research Fund-K U Leuven; Agency for Innovation by Science and Technology; Stichting tegen Kanker; Clinical Research Fund-University Hospitals Leuven; and Belgian Cancer Plan, Ministery of Health.

Long-Term Results of Congenital Aortic Stenosis Treatment in the Era of Percutaneous Balloon Valvuloplasty: Up to 33 Years Follow-Up.

Background The goal of this study was to evaluate long-term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children. Methods and Results A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow-up study. The resulting follow-up time reached a median of 18.5 (interquartile range, 12.2-25.1) years. Successful BVPL was defined by residual Doppler gradient <70/40 (systolic/mean) mm Hg. The primary end point was death; secondary end points included any valve reintervention, balloon revalvuloplasty, any aortic valve surgery, and aortic valve replacement, respectively. BVPL effectively reduced the peak and mean gradient both immediately and at the latest follow-up (P<0.001). There was significant procedure-related progression of aortic insufficiency (P<0.001). Higher aortic annulus z score was predictive for severe aortic regurgitation (P<0.05) and lower z score for insufficient gradient reduction (P<0.05). The actuarial probability of survival/survival free from any valve reintervention was 89.9%/59.9%, 85.9%/35.2%, and 82.0%/26.7% at 10, 20, and 30 years after first BVPL, respectively. Left ventricular dysfunction or arterial duct dependency as the indication for BVPL was predictive of both worse survival and survival free from any reintervention (P<0.001). Lower aortic annulus z score and lower balloon-to-annulus ratio were predictive of a need for revalvuloplasty (P<0.001). Conclusions Percutaneous BVPL provides good initial palliation. In patients with hypoplastic annuli and left ventricular or mitral valve comorbidity, the results are less favorable.

Pregnancy Termination and Postnatal Major Congenital Heart Defect Prevalence After Introduction of Prenatal Cardiac Screening.

Importance: Prenatal cardiac screening of the first and second trimesters has had a major impact on postnatal prevalence of congenital heart defects (CHDs), rates of termination of pregnancy (TOP), and outcomes among children born alive with CHDs. Objective: To examine the prenatal and postnatal incidence of major CHDs (ie, necessitating intervention within the first year of life), detection rate trends, rates of TOP, and the association of cardiac screening with postnatal outcomes. Design, Settings, and Participants: In this cross-sectional study, 3827 fetuses with antenatally diagnosed major CHDs in the Czech Republic (population 10.7 million) between 1991 and 2021 were prospectively evaluated with known outcomes and associated comorbidities. Prenatal and postnatal prevalence of CHD in an unselected population was assessed by comparison with a retrospective analysis of all children born alive with major CHDs in the same period (5454 children), using national data registry. Data analysis was conducted from January 1991 to December 2021. Main Outcomes and Measures: Prenatal detection and postnatal prevalence of major CHDs and rate of TOPs in a setting with a centralized health care system over 31 years. Results: A total of 3 300 068 children were born alive during the study period. Major CHD was diagnosed in 3827 fetuses, of whom 1646 (43.0%) were born, 2069 (54.1%) resulted in TOP, and 112 (2.9%) died prenatally. The prenatal detection rate increased from 6.2% in 1991 to 82.8% in 2021 (P < .001). Termination of pregnancy decreased from 70% in 1991 to 43% (P < .001) in 2021. Of 627 fetuses diagnosed in the first trimester (introduced in 2007), 460 were terminated (73.3%). Since 2007, of 2066 fetuses diagnosed in the second trimester, 880 (42.6%) were terminated, resulting in an odds ratio of 3.6 (95% CI, 2.8-4.6; P < .001) for TOP in the first trimester compared with the second trimester. Postnatal prevalence of major CHDs declined from 0.21% to 0.14% (P < .001). The total incidence (combining prenatal detection of terminated fetuses with postnatal prevalence) of major CHD remained at 0.23% during the study period. Conclusions and Relevance: In this cross-sectional study, the total incidence of major CHD did not change significantly during the 31-year study period. The prenatal detection of major CHD approached 83% in the current era. Postnatal prevalence of major CHD decreased significantly due to early TOPs and intrauterine deaths. The introduction of first trimester screening resulted in a higher termination rate in the first trimester but did not revert the overall decreasing trend of termination for CHDs in general.

Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.

BACKGROUND: Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. METHODS AND RESULTS: We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1-21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age < 20 weeks, ventricular rate ≤ 50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥ 1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4 ± 2.9 versus 24.9 ± 4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. CONCLUSIONS: Risk factors associated with a poor outcome were gestation < 20 weeks, ventricular rate ≤ 50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

Impact of liver fibrosis and nodules formation on hemodynamics in young adults after total cavopulmonary connection. A magnetic resonance study.

Background: The aim of this study was to analyze the relation between the hepatic fibrosis markers, liver morphology and hemodynamics assessed by magnetic resonance imaging (MRI) after total cavopulmonary connection (TCPC). Materials and methods: Adult patients after TCPC performed in childhood between 1993 and 2003 are the subjects of this observational study. The follow-up protocol consisted of clinical and echocardiographic examination, liver elastography, cardiopulmonary exercise test, MRI hemodynamics and liver morphology assessment and direct enhanced liver fibrosis (ELF) test. Results: The cohort consisted of 39 patients (46% female) with a median age at study 26 (IQR 23-28) years and interval from TCPC 21 (IQR 20-23) years. There was no correlation between ELF test and any MRI variables, but procollagen III amino-terminal peptide (PIIINP), a single component of ELF test, correlated significantly with ventricular end-diastolic volume (r = 0.33; p = 0.042) and inferior vena cava flow (r = 0.47; p = 0.003). Fifteen (38%) patients with liver nodules had compared to other 24 patients higher end-diastolic volume (ml/m2) 102.8 ± 20.0 vs. 88.2 ± 17.7; p = 0.023, respectively. PIIINP correlated significantly with inferior vena cava flow (r = 0.56; p = 0.030) and with end-diastolic volume (r = 0.53; p = 0.043), but only in patients with liver nodules. Conclusion: Gradual progression of liver fibrosis, particularly hepatic arterialization caused by liver nodules formation, increases inferior vena cava flow and subsequent ventricular volume overload may further compromise single ventricle functional reserve in adult patients after TCPC.

Shortening fraction of the right ventricle: a comparison between euploid and trisomy 21 fetuses at week 11 to week 13 and 6 days of gestation.

OBJECTIVES: This study was designed to compare the first trimester shortening fraction of the right ventricle (SFRV) values between euploid fetuses and fetuses with trisomy 21. METHODS: SFRV was measured in 58 first trimester fetuses between September 2008 and February 2010. The stored M-mode images were used to obtain the right ventricular diastolic diameter (RVDD) and right ventricular systolic diameter (RVSD) measurements offline. RESULTS: The SFRV values were found to be significantly greater in the 9 fetuses with trisomy 21 as compared to the group of 49 euploid fetuses (mean: 48.6 mm; range: 36-56.25 mm vs mean: 34.11 mm; range: 22.73-43.48 mm) (p < 0.0001). The RVDD measurements were also found to be significantly greater in the fetuses with trisomy 21 than in the euploid fetuses (mean: 3.08 mm; range: 2.2-4.7 mm vs mean: 2.54 mm; range: 1.9-3.6 mm) (p = 0.03).There was no difference in the RVSD measurements between the two groups [mean: 1.56 mm; range: 1.2-2.3 mm (trisomy 21 fetuses) vs mean: 1.67 mm; range: 1.3-2.4 mm (euploid fetuses)] (p = 0.17). CONCLUSIONS: The SFRV values in fetuses with trisomy 21 appear to be significantly greater than in the euploid fetuses. The RVDD also appears to be greater in fetuses with trisomy 21 than in the euploid fetuses.

Spectrum of postmortem autopsy findings in native and surgically corrected hearts with congenital malformations: a 10-year single-center experience.

OBJECTIVES: We reviewed a spectrum of congenital heart defects assessed in our center between 1/2010 and 4/2020, evaluated their gross anatomy, assessed the age distribution, evaluated performed surgical procedures, and correlated gross and ultrasound findings. METHODS: All necroptic cases and explanted hearts that underwent specialized cardiac autopsy were included in this study. Autopsy findings including gross description of congenital heart defects together with echocardiographic findings were retrospectively assessed. In surgically corrected hearts, the operation records were included as well. All congenital heart defects and surgical procedures were subclassified into main and additional category. RESULTS: The study included 92 necroptic cases of live-born children, 7 stillbirths, 2 cases of young adults, 50 induced abortions, and 5 explanted hearts, with median age 36 weeks. The most frequently encountered leading congenital heart defects were hypoplastic left heart syndrome, aortic stenosis, septal defects, or persistent arterial trunk. Fifty-one patients underwent surgical repair represented mainly by valvuloplasties, aortoplasty, and procedures leading to univentricular circulation. In the native hearts, 4 postnatal and 16 abortion/stillbirth cases showed discordance between gross and sonographic findings, mainly attributed to missed ventricular septal defect. Gestational age of the discordant group was significantly lower compared to the concordant group (P= .007). CONCLUSIONS: Autopsy continues to provide essential information about the morphology of congenital heart defects. However, the encountered congenital heart defects were usually complex, often surgically corrected or evaluated as a result of induced abortion or still birth. Cardiac autopsy therefore places high demands on pathologists with regards to proper gross heart assessment. It is also an invaluable part of quality control in prenatal cardiology.

Biallelic loss-of-function variants in PLD1 cause congenital right-sided cardiac valve defects and neonatal cardiomyopathy.

Congenital heart disease is the most common type of birth defect, accounting for one-third of all congenital anomalies. Using whole-exome sequencing of 2718 patients with congenital heart disease and a search in GeneMatcher, we identified 30 patients from 21 unrelated families of different ancestries with biallelic phospholipase D1 (PLD1) variants who presented predominantly with congenital cardiac valve defects. We also associated recessive PLD1 variants with isolated neonatal cardiomyopathy. Furthermore, we established that p.I668F is a founder variant among Ashkenazi Jews (allele frequency of ~2%) and describe the phenotypic spectrum of PLD1-associated congenital heart defects. PLD1 missense variants were overrepresented in regions of the protein critical for catalytic activity, and, correspondingly, we observed a strong reduction in enzymatic activity for most of the mutant proteins in an enzymatic assay. Finally, we demonstrate that PLD1 inhibition decreased endothelial-mesenchymal transition, an established pivotal early step in valvulogenesis. In conclusion, our study provides a more detailed understanding of disease mechanisms and phenotypic expression associated with PLD1 loss of function.

Left ventricle shortening fraction: a comparison between euploid and trisomy 21 fetuses in the first trimester.

OBJECTIVES: Measurement of the shortening fraction of the left ventricle (SFLV) is an objective way to assess systolic performance. The aim of the study was to compare first trimester SFLV values in euploid fetuses to those in fetuses with trisomy 21. METHODS: We measured SFLV in 56 fetuses from 11 weeks to 13 weeks 6 days. The left ventricular diastolic diameter (LVDD) and left ventricular systolic diameter (LVSD) were measured offline, and SFLV was calculated. The data were analyzed using Mann-Whitney U test. RESULTS: We found a significant difference in the SFLV measurements between the group of 49 euploid fetuses and the 7 fetuses with trisomy 21 [38.00 (95% CI: 33.72-42.27) vs 49.93 (95% CI: 43.72-56.13)] (p < 0.05). There was also a significant difference in the nuchal translucency measurements between the two groups: 1.78 mm (95% CI: 1.08-2.48 mm) in the euploid population versus 5.06 mm (95% CI: 3.61-6.71 mm) in the fetuses with trisomy 21 (p < 0.05). There were no significant differences between the group of euploid fetuses and the group of trisomy 21 fetuses in the following parameters: CRL (chorionic villus sampling), LVDD and LVSD. CONCLUSIONS: SFLV is a well-defined, simple measurement of systolic function of the fetal myocardium. SFLV values in fetuses with trisomy 21 appear to be significantly higher than in euploid fetuses.

Predictors of left ventricular remodelling and failure in right ventricular pacing in the young.

AIMS: To identify risk factors for left ventricular (LV) dysfunction in right ventricular (RV) pacing in the young. methods and results: Left ventricular function was evaluated in 82 paediatric patients with either non-surgical (n = 41) or surgical (n= 41) complete atrioventricular block who have been 100% RV paced for a mean period of 7.4 years. Left ventricular shortening fraction (SF) decreased from a median (range) of 39 (24-62)% prior to implantation to 32 (8-49)% at last follow-up (P < 0.05). Prevalence of a combination of LV dilatation (LV end-diastolic diameter >+2z-values) and dysfunction (SF < 0.26) was found to increase from 1.3% prior to pacemaker implantation to 13.4% (11/82 patients) at last follow-up (P = 0.01). Ten of these 11 patients had progressive LV remodelling and 8 of 11 were symptomatic. The only significant risk factor for the development of LV dilatation and dysfunction was the presence of epicardial RV free wall pacing (OR = 14.3, P < 0.001). Other pre-implantation demographic, diagnostic, and haemodynamic factors including block aetiology, pacing variables, and pacing duration did not show independent significance. CONCLUSION: Right ventricular pacing leads to pathologic LV remodelling in a significant proportion of paediatric patients. The major independent risk factor is the presence of epicardial RV free wall pacing, which should be avoided whenever possible.

Differential effects of the site of permanent epicardial pacing on left ventricular synchrony and function in the young: implications for lead placement.

AIMS: To analyse left ventricular (LV) synchrony and function with respect to the epicardial pacing site in the young. METHODS AND RESULTS: Left ventricular function and synchrony (M-mode, speckle tracking) were evaluated during mid-term follow-up in 32 children with complete non-surgical (n = 15) or surgical (n = 17) atrioventricular block (structural heart disease in 21/32) paced from LV apex (n = 19), right ventricular (RV) apex (n = 7), and RV free wall (n = 6), respectively. Data are in the following order: LV apical, RV apical, and RV free wall pacing. Septal to posterior wall motion delay (SPWMD) = median 0, 69, and 136 ms (P < 0.001), septal to lateral mechanical delay = 54 +/- 29, 73 +/- 24, and 129 +/- 70 ms (P = 0.001), apical to basal mechanical delay = 96 +/- 37, 106 +/- 50, and 79 +/- 18 ms (P NS), and LV ejection fraction (LVEF) = 57 +/- 9, 49 +/- 12, and 33 +/- 10% (P < 0.001), respectively. Left ventricular ejection fraction correlated negatively with SPWMD (R(2) = 0.454, P < 0.001) and septal to lateral mechanical delay (R(2) = 0.320, P < 0.001) but not with apical to basal mechanical delay. Right ventricular free wall pacing (P = 0.014) and SPWMD (P = 0.044) were negative multivariable predictors of LVEF. CONCLUSION: Compared with other sites, LV apical pacing preserves septal to lateral LV synchrony and systolic function and may be the preferred epicardial pacing site in the young.

Long-term (up to 20 years) results of percutaneous balloon angioplasty of recurrent aortic coarctation without use of stents.

AIMS: To assess the efficacy, safety, and long-term results of the balloon angioplasty of recoarctation. METHODS AND RESULTS: The angioplasty was performed in 99 consecutive patients aged 36 days to 32.6 years (median 268 days). Recoarctation to descending aorta diameter ratio increased from 0.44 (0.35/0.50) to 0.66 (0.57/0.77), P < 0.001. Systolic gradient was reduced from 34.0 (26.0/44.75) to 15.0 (8.25/27.0) mmHg, P < 0.001. In seven patients (7.1%) the procedure was ineffective. One patient (1%) with heart failure died within 24 h after a successful angioplasty and in another (1%) an intimal abruption necessitated surgical revision. The follow-up ranged up to 20.7 years (median 8.1 years). Actuarial probability of survival 20.7 years after the procedure was 0.91, and of reintervention-free survival was 0.44. Older age at the angioplasty was associated with a higher incidence of reinterventions (hazard ratio 1.057; 95% confidence interval 1.012-1.103; P = 0.010). The type of surgery and the recoarctation anatomy did not influence the outcome. In 69 patients aneurysm formation was studied by high-sensitive methods with only one positive finding per 462 patient-years. CONCLUSION: Angioplasty is safe and effective regardless of the type of surgery used and the recoarctation anatomy. Older age at the angioplasty is associated with a higher incidence of reinterventions.