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Anesthetic management for cesarean delivery in a patient with uncorrected pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries.

Int J Obstet Anesth. 2018 Nov;36:125-129

Authors: Gang SP, Fang KY, Ma Y, Zhang FX, Xiang DK, Liu XL, Wang RP, Chen DD, Ma XW

Abstract
Pulmonary atresia witha ventricular septal defect and major aortopulmonary collateral arteries is an extremely rare congenital disorder characterized by a high risk of maternal mortality. We present the case of a 24-year-old primigravid woman with uncorrected pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arterieswho presented at 35+5 weeks' gestation. Based on the pathophysiology of the congenital cardiac lesion, cesarean delivery was performed under epidural anesthesia under management by a multidisciplinary team. This report highlights the anesthesia management of a rare uncorrected congenital cardiac lesion for cesarean delivery.

PMID: 30054110 [PubMed - indexed for MEDLINE]

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22q11.2 deletion detected by in situ hybridization in Mexican patients with velocardiofacial syndrome-like features.

Colomb Med (Cali). 2018 Sep 30;49(3):219-222

Authors: Ramírez-Velazco A, Rivera H, Vásquez-Velázquez AI, Aguayo-Orozco TA, Delgadillo-Pérez S, Domínguez MG

Abstract
Introduction: Deletion 22q11.2 occurs in 1:4,000-1:6,000 live births while 10p13p14 deletion is found in 1:200,000 newborns. Both deletions have similar clinical features such as congenital heart disease and immunological anomalies.
Objective: We looked for a 22q11.2 deletion in Mexican patients with craniofacial dysmorphisms suggestive of DiGeorge or velocardiofacial syndromes and at least one major phenotypic feature (cardiac anomaly, immune deficiency, palatal defects or development delay).
Methods: A prospective study of 39 patients recruited in 2012-2015 at the Instituto Mexicano del Seguro Social at Guadalajara, Mexico. The patients with velocardiofacial syndrome-like features or a confirmed tetralogy of Fallot (TOF) or complex cardiopathy were studied by G-banding and fluorescence in situ hybridization (FISH) with a dual TUPLE1(HIRA)/ARSA or TUPLE1(22q11)/22q13(SHANK3) probe, six patients without the 22q11.2 deletion (arbitrarily selected) were tested with the dual DiGeorge II (10p14)/D10Z1 probe.
Results: Twenty-two patients (7 males and 15 females) had the 22q11.2 deletion and 17/39 did not have it; no patient had a 10p loss. Among the 22 deleted patients, 19 had congenital heart disease (mostly TOF). Twelve patients without deletion had heart defects such as TOF (4/12), isolate ventricular septal defect (2/12) or other disorders (6/12).
Conclusion: In our small sample about ~56% of the patients, regardless of the clinical diagnosis, had the expected 22q11.2 deletion. We remark the importance of early cytogenetic diagnosis in order to achieve a proper integral management of the patients and their families.

PMID: 30410196 [PubMed - indexed for MEDLINE]

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Pol Merkur Lekarski. 2019 Feb 28;46(272):60-63

Authors: Okólska M, Róg B, Sałapa K, Tomkiewicz-Pająk L

Abstract
The bicuspid aortic valve (BAV) is a congenital heart disease.
AIM: The aim of study was to evaluate the assessment of atherosclerosis risk factors in adult patients with bicuspid aortic valve compared against 30 healthy, age and sex matched volunteers.
MATERIALS AND METHODS: Thirty patients (22 males, 8 females) at an average age of 27.00 ±10.6 years were compared against thirty healthy, age and sex matched volunteers. The arterial blood pressure, lipid profile, fasting glucose, inflammation markers and the lifestyle factors were analysed.
RESULTS: Thirty patients (22 males, 8 females) at an average age of 27.00 ±10.6 years were compared against thirty healthy, age and sex matched volunteers. The arterial blood pressure, lipid profile, fasting glucose, inflammation markers and the lifestyle factors were analysed.
CONCLUSIONS: The patients with bicuspid aortic valve have higher glucose and fibrinogen levels and they need systematic checking to reduce cardiovascular risk factors.

PMID: 30830889 [PubMed - in process]

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Obstetric and cardiac outcomes in women with Marfan syndrome and an aortic root diameter ≤ 45mm.

Eur J Obstet Gynecol Reprod Biol. 2018 Nov;230:68-72

Authors: Minsart AF, Mongeon FP, Laberge AM, Morin F, Dore A, Leduc L

Abstract
OBJECTIVE: To assess obstetric and aortic outcomes in women with Marfan Syndrome according to aortic root diameter, in view of recommendations for caesarean delivery when the aortic root diameter is ≥40 mm in the 2010 American guidelines versus >45 mm in the 2011 European guidelines.
STUDY DESIGN: In this retrospective cohort study conducted at Sainte-Justine Mother and Child Tertiary Hospital, 27 pregnancies in 20 women with Marfan Syndrome as defined by the international criteria, were followed prospectively between 1994 and 2017, after excluding women with prior aortic surgery. Obstetric and aortic outcomes were compared in 2 groups according to aortic root diameter: < 40 mm (21 pregnancies) and 40-45 mm (6 pregnancies).
RESULTS: 21/27 women had a vaginal delivery. The caesarean section rate was 23.8% and 16.7% in women with diameter <40 mm and 40-45 mm respectively (p-value = 1), and perinatal outcome was similar across groups. Two women with a prepregnancy aortic root diameter <40 mm developed an acute type B dissection during the third trimester. Both had a family history of aortic dissection.
CONCLUSIONS: Vaginal delivery with rigorous pain control and avoidance of Valsalva maneuver may be safely considered in women with Marfan Syndrome and an aortic root diameter ≤45 mm. The risk of type B aortic dissection during pregnancy is hard to predict. Other factors such as family history of dissection and descending aorta size may play an important role, and this may modify our counselling.

PMID: 30243228 [PubMed - indexed for MEDLINE]

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Cardiopulmonary Exercise Testing-A Valuable Tool, Not Gatekeeper When Referring Patients With ACHD for Transplant Evaluation.

World J Pediatr Congenit Heart Surg. 2019 Mar 04;:2150135118825263

Authors: Menachem JN, Reza N, Mazurek JA, Burstein D, Birati EY, Fox A, Kim YY, Molina M, Partington SL, Tanna M, Tobin L, Wald J, Goldberg LR

Abstract
INTRODUCTION:: Treatment of patients with adult congenital heart disease (ACHD) with advanced therapies including heart transplant (HT) is often delayed due to paucity of objective prognostic markers for the severity of heart failure (HF). While the utility of Cardiopulmonary Exercise Testing (CPET) in non-ACHD patients has been well-defined as it relates to prognosis, CPET for this purpose in ACHD is still under investigation.
METHODS:: We performed a retrospective cohort study of 20 consecutive patients with ACHD who underwent HT between March 2010 and February 2016. Only 12 of 20 patients underwent CPET prior to transplantation. Demographics, standard measures of CPET interpretation, and 30-day and 1-year post transplantation outcomes were collected.
RESULTS:: Patient Characteristics. Twenty patients with ACHD were transplanted at a median of 40 years of age (range: 23-57 years). Of the 12 patients who underwent CPET, 4 had undergone Fontan procedures, 4 had tetralogy of Fallot, 3 had d-transposition of the great arteries, and 1 had Ebstein anomaly. Thirty-day and one-year survival was 100%. All tests included in the analysis had a peak respiratory quotient _1.0. The median peak oxygen consumption per unit time (_VO2) for all diagnoses was 18.2 mL/kg/min (46% predicted), ranging from 12.2 to 22.6.
CONCLUSION:: There is a paucity of data to support best practices for patients with ACHD requiring transplantation. While it cannot be proven based on available data, it could be inferred that outcomes would have been worse or perhaps life sustaining options unavailable if providers delayed referral because of the lack of attainment of CPET-specific thresholds.

PMID: 30832541 [PubMed - as supplied by publisher]

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Clinical Evaluation of Exercise Capacity in Adults with Systemic Right Ventricle.

Tex Heart Inst J. 2019 Feb;46(1):14-20

Authors: Rog B, Salapa K, Okolska M, Dluzniewska N, Werynski P, Podolec P, Tomkiewicz-Pajak L

Abstract
The right ventricle provides systemic circulation in individuals with congenitally corrected transposition of the great arteries (CCTGA) and in those with complete transposition who have had an atrial switch repair (DTGA). The aim of this study was to evaluate how the systemic right ventricle adapts to increased workload and oxygen demand during exercise. From November 2005 through December 2015, 3,358 adult patients with congenital heart disease were treated at our institution; we identified 48 (26 females, 22 males; median age, 25.4 ± 8.1 yr) who met the study criteria; 37 had DTGA and atrial switch repair, and 11 had CCTGA. We studied their echocardiographic and cardiopulmonary exercise test results. A control group consisted of 29 healthy sex- and age-matched volunteers. On exercise testing, oxygen uptake at anaerobic threshold, peak oxygen uptake, peak heart rate, and percentage of maximal heart rate were significantly lower in the group with systemic right ventricle than in the control group (all P <0.001); in contrast, the peak ventilatory equivalent for carbon dioxide was higher in the study group (P=0.013). Impaired systemic right ventricular function reduced peak oxygen uptake. The peak heart rate was lower in the CCTGA group than in the DTGA group. Our results indicate that reduced exercise capacity is related to impaired systemic right ventricular function, severe tricuspid valve regurgitation, and chronotropic incompetence. There was no correlation between cardiopulmonary exercise test results and time after surgery. Chronotropic efficiency is lower in individuals with CCTGA than in those with DTGA.

PMID: 30833832 [PubMed - in process]

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Misclassification of bicuspid aortic valves is common and varies by imaging modality and patient characteristics.

Echocardiography. 2019 Mar 04;:

Authors: Cramer PM, Prakash SK

Abstract
BACKGROUND: Bicuspid aortic valve (BAV) is the most prevalent adult congenital heart defect. BAV causes lifelong progressive disease that can be prevented by early diagnosis and long-term surveillance, but may be compromised by misclassification of valve morphology.
METHODS: The study population was derived from the UTHealth Bicuspid Aortic Valve Registry, which includes serial images on more than 200 participants over a mean follow-up interval of 2.8 years. We abstracted descriptions of aortic valve morphology from transthoracic or transesophageal echocardiography, computed tomography angiography, and magnetic resonance angiography reports. We used chi-square and t tests to determine associations between reported valve morphologies (definitely bicuspid, possibly bicuspid, tricuspid, or uncertain) and clinical characteristics and assessed image quality using a validated tool.
RESULTS: About 40% of participants were misclassified in at least one imaging report. The mean interval between misclassification and correct diagnosis was 22 months. TEE, MR and CT were more sensitive than TTE and successfully reclassified 20% of participants, but were only used in 14% of patients. Misclassification was associated with age, the extent of valve calcification and image quality, but was not significantly associated with aortic regurgitation, gender, or cusp configuration.
CONCLUSION: Misclassification of BAV is prevalent, frequently leads to delayed diagnosis, and is more likely to occur in the most severely affected cases. TEE, CT and MR may increase diagnostic accuracy in up to half of BAV cases but are underutilized. Additional studies are needed to determine if misclassification of BAV patients leads to increased long-term morbidity and mortality.

PMID: 30834578 [PubMed - as supplied by publisher]

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Predictors of extracorporeal membrane oxygenation support after surgery for adult congenital heart disease in children's hospitals.

Congenit Heart Dis. 2019 Mar 05;:

Authors: Dolgner SJ, Krieger EV, Wilkes J, Bratton SL, Thiagarajan RR, Barrett CS, Chan T

Abstract
OBJECTIVE: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals.
DESIGN: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups: ECMO-free survival, requiring ECMO, and DWE. Univariate analyses were performed, and multinomial logistic regression models were constructed examining ECMO and DWE as independent outcomes.
SETTING: Tertiary care children's hospitals.
RESULTS: A total of 4665 adult patients underwent ACHD surgery in 39 children's hospitals with 51 (1.1%) patients requiring ECMO and 64 (1.4%) patients experiencing DWE. Of the 51 ECMO patients, 34 (67%) died. Increasing patient age, surgical complexity, diagnosis of single ventricle heart disease, preoperative hospitalization, and the presence of noncardiac complex chronic conditions (CCC) were risk factors for both outcomes. Additionally, low and medium hospital ACHD surgical volume was associated with an increased risk of DWE in comparison with ECMO.
CONCLUSIONS: There are overlapping but separate risk factors for ECMO support and DWE among adults undergoing congenital heart surgery in pediatric hospitals.

PMID: 30835967 [PubMed - as supplied by publisher]

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Authors' Reply.

J Am Soc Echocardiogr. 2017 10;30(10):1041

Authors: Baumgartner H, Hung J

PMID: 28864151 [PubMed - indexed for MEDLINE]

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Cardiac Magnetic Resonance Evaluation of Pulmonary Transit Time and Blood Volume in Adult Congenital Heart disease.

J Magn Reson Imaging. 2019 Mar 05;:

Authors: Ait Ali L, Aquaro GD, Peritore G, Ricci F, De Marchi D, Emdin M, Passino C, Festa P

Abstract
BACKGROUND: Management of adults with repaired congenital heart disease (CHD) is still challenging. Heart failure secondary to residual anatomical sequels or arrhythmic events is not rare in this population. MRI has emerged as an accurate tool to quantify pulmonary transit time (PTT) of intravenous contrast agents and pulmonary blood volume (PBV).
PURPOSE: To determine the relationship between PTT, and conventional indexes of ventricular dysfunction and heart failure in a cohort of adults with CHD and to assess its association with adverse outcomes.
STUDY TYPE: Retrospective.
SUBJECTS: 89 adult CHD patients (56 males, age 34 ± 11 years) and 14 age- and sex-matched healthy subjects.
FIELD STRENGTH/SEQUENCE: First-pass perfusion and standard sequences for ventricular volumes and function and flow analysis at 1.5T.
ASSESSMENT: PTT was calculated as the time required for a bolus of contrast agent to pass from the right ventricle to the left atrium, expressed both in seconds (PTTS) and number of heartbeats (PTTB). The pulmonary blood volume index (PBVI) was measured by the product of PTTB and the pulmonary artery stroke volumes.
STATISTICAL TESTS: Student's independent t-test analysis of variance (ANOVA) and Mann-Whitney nonparametric; Pearson's or Spearman's correlation; Kaplan-Meier method.
RESULTS: PTTS and PTTB were significantly higher in patients than in controls (7.6 ± 3 vs. 5.6 ± 1.2 sec, P = 0.01 and 8 ± 3 vs. 6 ± 1 bpm, P = 0.01, respectively). PTTS showed negative correlation with left ventricle ejection fraction (LVEF) and cardiac index (CI) (r = -0.3, P = 0.004, and r = -0.4, P < 0.001, respectively) as well as with left ventricle and atrial volumes. By Kaplan-Meier survival analysis, PTTB >8 bpm was associated with significant increased risk of adverse outcome at mid-term follow-up. Moreover, patients with both increased PTTB and PBV have higher amino-terminal portion of the prohormone brain natriuretic peptide (NT-proBNP) and lower LVEF.
DATA CONCLUSION: PTT is prolonged in adult CHD in comparison with healthy subjects, likely reflecting reduced CI and ventricular dysfunction.
LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2019.

PMID: 30838716 [PubMed - as supplied by publisher]

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