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Objective: Second pregnancies are usually less complicated than first pregnancies, and have a better outcome in terms of fetal growth. We studied a group of women with heart disease to assess whether their second pregnancy was less complicated and resulted in a larger baby.Design: Retrospective case control study.Setting: Tertiary referral academic obstetric unit.Population: First and second pregnancies in 77 women with congenital and acquired heart disease and in 154 control women were identified.Methods: Data were collected from medical and obstetric records.Main Outcome Measures: Cardiac complications, obstetric complications, intra-partum events, birthweight and perinatal complications.Results: The rate of obstetric complication was greater in first pregnancies in both the heart disease and the control groups (38% versus 26%, cf. 20% versus 17%). In the heart disease group, the rate of cardiac complications was similar in first and second pregnancies (9% versus 6%). Overall, significantly more perinatal complications were seen in the heart disease group, with no significant difference between first and second pregnancies (36% versus 27%, cf. 14% versus 12%). Median birthweight was significantly higher in second pregnancies in the control group (3308 versus 3519 g P < 0.001), but not significantly different between pregnancies in the heart disease group (3014 versus 3133 g, P = 0.19).Conclusions: This case control study demonstrates that women with mild to moderate heart disease have similar pregnancy outcomes in consecutive pregnancies. However, while the median birthweight was higher in the control second pregnancies, it was not increased in the women with heart disease.Tweetable Abstract: A study of women with heart disease to assess whether their second pregnancy was less complicated. [ABSTRACT FROM AUTHOR]

Objective To report outcomes in a recent series of pregnancies in women with Marfan syndrome ( MFS). Design Retrospective case note review. Setting Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals). Sample Twenty-nine pregnancies in 21 women with MFS between 1995 and 2010. Methods Multidisciplinary review of case records. Main outcome measures Maternal and neonatal mortality and morbidity of patients with MFS and healthy controls. Results There were no maternal deaths. Significant cardiac complications occurred in five pregnancies (17%): one woman experienced a type-A aortic dissection; two women required cardiac surgery within 6 months of delivery; and a further two women developed impaired left ventricular function during the pregnancy. Women with MFS were also more likely to have obstetric complications ( OR 3.29, 95% CI 1.30-8.34), the most frequent of which was postpartum haemorrhage ( OR 8.46, 95% CI 2.52-28.38). There were no perinatal deaths, although babies born to mothers with MFS were delivered significantly earlier than those born to the control group (median 39 versus 40 weeks of gestation, Mann-Whitney U-test, P = 0.04). These babies were also significantly more likely to be small for gestational age (24% in the MFS group versus 6% in the controls; OR 4.95, 95% CI 1.58-15.55). Conclusions Pregnancy in women with MFS continues to be associated with significant rates of maternal, fetal, and neonatal complications. Effective pre-pregnancy counselling and meticulous surveillance during pregnancy, delivery, and the puerperium by an experienced multidisciplinary team are warranted for women with MFS. [ABSTRACT FROM AUTHOR]

Please cite this paper as: Curry R, Fletcher C, Gelson E, Gatzoulis M, Woolnough M, Richards N, Swan L, Steer P, Johnson M. Pulmonary hypertension and pregnancy-a review of 12 pregnancies in nine women. BJOG 2012;119:752-761. Objective To report outcomes in a recent series of pregnancies in women with pulmonary hypertension (PH). Design Retrospective case note review. Setting Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals). Sample Twelve pregnancies in nine women with PH between 1995 and 2010. Methods Multidisciplinary review of case records. Main outcome measures Maternal and neonatal mortality and morbidity. Results There were two maternal deaths (1995 and 1998), one related to pre-eclampsia and one to arrhythmia. Maternal morbidity included postpartum haemorrhage (five cases), and one post-caesarean evacuation of a wound haematoma. There were no perinatal deaths, nine live births and three first-trimester miscarriages. Mean birthweight was 2197 g, mean gestational age was 34 weeks (range 26-39), and mean birthweight centile was 36 (range 5-60). Five babies required admission to the neonatal intensive care unit, but were all eventually discharged home. All women were delivered by caesarean section (seven elective and two emergency deliveries), under general anaesthetic except for one emergency and one elective caesarean performed under regional block. Conclusions Maternal and fetal outcomes for women with PH may be improving. However, the risk of maternal mortality remains significant, so that early and effective counselling about contraceptive options and pregnancy risks should continue to play a major role in the management of such women when they reach reproductive maturity. [ABSTRACT FROM AUTHOR]

Background Establishing surgical criteria for aortic valve replacement (AVR) in severe aortic regurgitation in young adults is challenging due to the lack of evidence-based recommendations. We studied indications for AVR in young adults with severe aortic regurgitation and their outcomes, as well as the relationship between presurgical echocardiographic parameters and postoperative left ventricular (LV) size, function, clinical events, and valve-related complications. Methods and Results Data were collected retrospectively on 172 consecutive adult patients who underwent AVR or repair for severe aortic regurgitation between 2005 and 2019 in a tertiary cardiac center (age at surgery 29 [22-41] years, 81% male). One-third underwent surgery before meeting guideline indications. Postsurgery, 65% achieved LV size and function normalization. LV ejection fraction showed no significant change from baseline. A higher presurgical LV end-systolic diameter correlated with a lack of LV normalization (odds ratio per 1-cm increase 2.81, P <0.01). The baseline LV end-systolic diameter cut-off for predicting lack of LV normalization was 43 mm. Pre- and postoperative LV dimensions and postoperative LV ejection fraction predicted clinical events during follow-up. Prosthetic valve-related complications occurred in 20.3% during an average 5.6-year follow-up. Freedom from aortic reintervention was 98%, 96.5%, and 85.4% at 1, 5, and 10 years, respectively. Conclusions Young adult patients with increased baseline LV end-systolic diameter or prior cardiac surgery are less likely to achieve LV normalization after AVR. Clinicians should carefully balance the long-term benefits of AVR against procedural risks and future interventions, especially in younger patients. Evidence-based criteria for AVR in severe aortic regurgitation in young adults are crucial to improve outcomes.

Background There are limited data about cardiac resynchronization therapy (CRT) in adult congenital heart disease. We aimed to assess early and late outcomes of CRT among patients with adult congenital heart disease. Methods and Results We retrospectively studied 54 patients with adult congenital heart disease (median age, 46 years; range, 18-73 years; 74% men) who received CRT implantation (biventricular paced >90%) between 2004 and 2017. Clinical and echocardiographic data were analyzed at baseline and early (mean±SD: 1.8±0.8 years) and late (4.7±0.8 years) follow-up after CRT. Compared with baseline, CRT was associated with significant improvement at early follow-up in New York Heart Association functional class, QRS duration, and cardiothoracic ratio ( P <0.05 for all); improvement in New York Heart Association class was sustained at late follow-up. Among patients with a systemic left ventricle (LV; n=39), there was significant increase in LV ejection fraction and reduction in LV end-systolic volume at early and late follow-up ( P <0.05 for both). For patients with a systemic right ventricle (n=15), there was a significant early but not late reduction in systemic right ventricular basal and longitudinal diameters. Eleven patients died, and 2 had heart transplantation unrelated to systemic ventricular morphological characteristics. Thirty-five patients (65%) responded positively to CRT, but only baseline QRS duration was predictive of a positive response. Conclusions CRT results in sustained improvement in functional class, systemic LV size, and function. Patients with a systemic LV and prolonged QRS duration, independent of QRS morphological characteristics, were most likely to respond to CRT.

The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success story of modern medicine. In the 1950s, survival of children born with CHD was only approximately 15%, whereas nowadays more than 90% of these children survive well into adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients with CHD over 60 years old. Many adult patients are afflicted by residual haemodynamic lesions and also face additional opportunities and/or challenges such as pregnancy, acquired heart disease, non-cardiac pathology etc., necessitating integrated care and all medical disciplines. We are faced with a "tsunami" in terms of adult CHD numbers, disease heterogeneity and complexity of work and interventions needed. We need to secure resources, welcome more people in our field, learn from "marching with our patients", and educate better patients, public and ourselves so that every single patient with CHD, born anywhere in the world, may reach their full life potential., (©2019 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Transthoracic echocardiography is the principal imaging modality for assessment of patients with atrioventricular septal defects. Three-dimensional echocardiography streamlines and simplifies data acquisition offering a unique realistic en-face display of heart valves and septal defects and enables accurate evaluation of the cardiac anatomy, dynamic, and function. We demonstrated an added value of three-dimensional echocardiography in assessment of an adult patient with atrioventricular septal defect and its advantages over conventional echocardiography., (© 2019 Wiley Periodicals, Inc.)

Background Atrial tachycardia ( AT ) is common in patients with adult congenital heart disease and is challenging to map and ablate. We used ultra-high-density mapping to characterize the AT mechanism and investigate whether substrate characteristics are related to ablation outcomes. Methods and Results A total of 50 AT s were mapped with ultra-high-density mapping in 23 procedures. Patients were followed up for up to 12 months. Procedures were classified to group A if there was 1 single AT induced (n=12) and group B if there were ≥2 AT s induced (n=11 procedures). AT mechanism per procedure was macro re-entry (n=10) and localized re-entry (n=2) in group A and multiple focal (n=6) or multiple macro re-entry (n=5) in group B. Procedure duration, low voltage area (0.05-0.5 mV), and low voltage area indexed for volume were higher in group B (159 [147-180] versus 412 [352-420] minutes, P<0.001, 22.6 [12.2-29.8] versus 54.2 [51.1-61.6] cm 2 , P=0.014 and 0.17 [0.12-0.21] versus 0.26 [0.23-0.27] cm 2 /mL, P=0.024 accordingly). Dense scar (<0.05 mV) and atrial volume were similar between groups. Acute success and freedom from arrhythmia recurrence were worse in group B (100% versus 77% P=0.009 and 11.3, CI 9.8-12.7 versus 4.9, CI 2.2-7.6 months, log rank P=0.004). Indexed low voltage area ≥0.24 cm 2 /mL could predict recurrence with 100% sensitivity and 77% specificity (area under the curve 0.923, P=0.007). Conclusions Larger low voltage area but not dense scar is associated with the induction of multiple focal or re-entry AT s, which are subsequently associated with longer procedure duration and worse acute and midterm clinical outcomes.

Background: The aim was to assess whether cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects hemodynamic stability, in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation, or aortic root dilatation., Methods and Results: This was a retrospective cohort study of women with repaired TOF with paired cardiovascular magnetic resonance scans before and after their first pregnancy (baseline RV end systolic volume index 49 mL/m 2 and RV end diastolic volume index 118 mL/m 2 ) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline cardiovascular magnetic resonance scan, time between follow-up of cardiovascular magnetic resonance scans, QRS duration, RV ejection fraction, and indexed RV end systolic and diastolic volume at baseline. Effect of pregnancy and time on parameters was assessed using mixed-effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions, or exercise data. There was an effect of pregnancy observed in both left ventricular end diastolic volume and left ventricular stroke volume, consistent with a sustained small increase in left ventricular stroke volume attributed to pregnancy (53-55 mL/m 2 )., Conclusions: Women with repaired TOF and with mild-to-moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that prepregnancy counseling is tailored to their individual clinical status., (© 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.)

Objective: Second pregnancies are usually less complicated than first pregnancies, and have a better outcome in terms of fetal growth. We studied a group of women with heart disease to assess whether their second pregnancy was less complicated and resulted in a larger baby., Design: Retrospective case control study., Setting: Tertiary referral academic obstetric unit., Population: First and second pregnancies in 77 women with congenital and acquired heart disease and in 154 control women were identified., Methods: Data were collected from medical and obstetric records., Main Outcome Measures: Cardiac complications, obstetric complications, intra-partum events, birthweight and perinatal complications., Results: The rate of obstetric complication was greater in first pregnancies in both the heart disease and the control groups (38% versus 26%, cf. 20% versus 17%). In the heart disease group, the rate of cardiac complications was similar in first and second pregnancies (9% versus 6%). Overall, significantly more perinatal complications were seen in the heart disease group, with no significant difference between first and second pregnancies (36% versus 27%, cf. 14% versus 12%). Median birthweight was significantly higher in second pregnancies in the control group (3308 versus 3519 g P < 0.001), but not significantly different between pregnancies in the heart disease group (3014 versus 3133 g, P = 0.19)., Conclusions: This case control study demonstrates that women with mild to moderate heart disease have similar pregnancy outcomes in consecutive pregnancies. However, while the median birthweight was higher in the control second pregnancies, it was not increased in the women with heart disease., Tweetable Abstract: A study of women with heart disease to assess whether their second pregnancy was less complicated., (© 2015 Royal College of Obstetricians and Gynaecologists.)

Objective: To report outcomes in a recent series of pregnancies in women with Marfan syndrome (MFS)., Design: Retrospective case note review., Setting: Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals)., Sample: Twenty-nine pregnancies in 21 women with MFS between 1995 and 2010., Methods: Multidisciplinary review of case records., Main Outcome Measures: Maternal and neonatal mortality and morbidity of patients with MFS and healthy controls., Results: There were no maternal deaths. Significant cardiac complications occurred in five pregnancies (17%): one woman experienced a type-A aortic dissection; two women required cardiac surgery within 6 months of delivery; and a further two women developed impaired left ventricular function during the pregnancy. Women with MFS were also more likely to have obstetric complications (OR 3.29, 95% CI 1.30-8.34), the most frequent of which was postpartum haemorrhage (OR 8.46, 95% CI 2.52-28.38). There were no perinatal deaths, although babies born to mothers with MFS were delivered significantly earlier than those born to the control group (median 39 versus 40 weeks of gestation, Mann-Whitney U-test, P = 0.04). These babies were also significantly more likely to be small for gestational age (24% in the MFS group versus 6% in the controls; OR 4.95, 95% CI 1.58-15.55)., Conclusions: Pregnancy in women with MFS continues to be associated with significant rates of maternal, fetal, and neonatal complications. Effective pre-pregnancy counselling and meticulous surveillance during pregnancy, delivery, and the puerperium by an experienced multidisciplinary team are warranted for women with MFS., (© 2014 The Authors BJOG An International Journal of Obstetrics and Gynaecology published by John Wiley & Sons Ltd on behalf of Royal College of Obstetricians and Gynaecologists.)

Objective: To report outcomes in a recent series of pregnancies in women with pulmonary hypertension (PH)., Design: Retrospective case note review., Setting: Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals)., Sample: Twelve pregnancies in nine women with PH between 1995 and 2010., Methods: Multidisciplinary review of case records., Main Outcome Measures: Maternal and neonatal mortality and morbidity., Results: There were two maternal deaths (1995 and 1998), one related to pre-eclampsia and one to arrhythmia. Maternal morbidity included postpartum haemorrhage (five cases), and one post-caesarean evacuation of a wound haematoma. There were no perinatal deaths, nine live births and three first-trimester miscarriages. Mean birthweight was 2197 g, mean gestational age was 34 weeks (range 26-39), and mean birthweight centile was 36 (range 5-60). Five babies required admission to the neonatal intensive care unit, but were all eventually discharged home. All women were delivered by caesarean section (seven elective and two emergency deliveries), under general anaesthetic except for one emergency and one elective caesarean performed under regional block., Conclusions: Maternal and fetal outcomes for women with PH may be improving. However, the risk of maternal mortality remains significant, so that early and effective counselling about contraceptive options and pregnancy risks should continue to play a major role in the management of such women when they reach reproductive maturity., (© 2012 The Authors BJOG An International Journal of Obstetrics and Gynaecology © 2012 RCOG.)

22q11 Deletion Syndrome (22q11DS) is a common microdeletion syndrome with multisystem expression. Phenotypic features vary with age, ascertainment, and assessment. We systematically assessed 78 adults (36 M, 42 F; mean age 31.5, SD 10.5 years) with a 22q11.2 deletion ascertained through an adult congenital cardiac clinic (n = 35), psychiatric-related sources (n = 39), or as affected parents of subjects (n = 4). We recorded the lifetime prevalence of features requiring attention, with 95% confidence intervals (CI) not overlapping zero. Subtle learning difficulties, hypernasality and facial gestalt were not included. We investigated ascertainment effects using non-overlapping subgroups ascertained with tetralogy of Fallot (n = 31) or schizophrenia (n = 31). Forty-three features met inclusion criteria and were present in 5% or more patients, including several of later onset (e.g., hypothyroidism, cholelithiasis). Number of features per patient (median 9, range 3-22) correlated with hospitalizations (P = 0.0002) and, when congenital features were excluded, with age (P = 0.02). Adjusting for ascertainment, 25.8% (95% CI, 9.5-42.1%) of patients had cardiac anomalies and 22.6% (95% CI, 7.0-38.2%) had schizophrenia. Ascertainment subgroups were otherwise similar in median number and prevalence of features. Non-characteristic features are common in 22q11DS. Adjusting for ascertainment effects is important. Many treatable conditions may be anticipated and features may accumulate over time. The results have implications for clinical assessment and management, genetic counseling and research into pathophysiological mechanisms., (Copyright 2005 Wiley-Liss, Inc)