scholarly journals Coarctation Stenting in a Rare Case with Congenitally Corrected Transposition of the Great Arteries and the Bicuspid Aortic Valve

Author(s):  
Zahra Khajali ◽  
Majid Maleki ◽  
Bahram Mohebbi ◽  
Maryam Aliramezany

Congenitally corrected transposition of the great arteries (cc-TGA) is a rare congenital abnormality that occurs in 1 per 33000 live births. This abnormality comprises nearly 0.05% of all congenital heart defects, with at least 90% of cc-TGA patients having associated cardiac defects; some of these associated defects are, however, very rare. In this case report, we describe a 22-year-old man who referred to our hospital for the evaluation of hypertension and cardiac murmurs. Via echocardiography and catheterization, the patient was finally diagnosed with cc-TGA, bicuspid aortic valve, and coarctation of the aorta. He underwent successful percutaneous transarterial coarctoplasty without any complications at early and 6 months’ follow-up visits.

2011 ◽  
Vol 29 (1) ◽  
pp. E13-E15 ◽  
Author(s):  
Paolo Giuseppe Pino ◽  
Amedeo Pergolini ◽  
Giordano Zampi ◽  
Angela Calicchia ◽  
Claudia Chialastri ◽  
...  

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Faysal Şaylık ◽  
Ferit Onur Mutluer ◽  
Aydın Tosu ◽  
Murat Selçuk

Double orifice mitral valve is a very rare congenital abnormality. Well known associations of this pathology with other congenital lesions point to a complex and central pathophysiological mechanism leading to a sequence of pathologies. These associations have long been realized and arbitrarily defined as Shone complex. We would like to present a 21-year-old patient with double orifice mitral valve associated with bicuspid aortic valve, with a brief review of the literature on possible central mechanisms leading to different subsets of congenital abnormalities involving these two.


2018 ◽  
Vol 2 (s1) ◽  
pp. 64-66
Author(s):  
Yeltay Rakhmanov ◽  
Paolo Enrico Maltese ◽  
Alessandra Zulian ◽  
Tommaso Beccari ◽  
Munis Dundar ◽  
...  

Abstract Coarctation of the aorta (CoA) is an inherited narrowing of the proximal descending thoracic aorta. Histological features include localized medial thickening and infolding with superimposed neointimal tissue. CoA is diagnosed by detection of a murmur or hypertension during routine examination. Typical clinical features are delayed or absent femoral pulses and difference in blood pressure between the arm and legs. These symptoms may appear in the first weeks of life or after the neonatal period. CoA accounts for 4-6% of all congenital heart defects and has a reported prevalence of about 4 per 10,000 live births. It is more common in males than females (59% vs 41%). This Utility Gene Test was developed on the basis of an analysis of the literature and existing diagnostic protocols. It is useful for confirming diagnosis, as well as for differential diagnosis, couple risk assessment and access to clinical trials.


2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Aureo Campos-Tello ◽  
Giancarlo A Valle ◽  
Felix A Revilla ◽  
Sara Rami­rez-Flores ◽  
Ãngel CuevaParra ◽  
...  

Author(s):  
Alessandro Verzini ◽  
Marta Bargagna ◽  
Guido Ascione ◽  
Alessandra Sala ◽  
Davide Carino ◽  
...  

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and it is responsible for an increased risk of developing aortic valve and ascending aorta complications. In case of mild to moderate BAV disease in patients undergoing supracoronary ascending aorta replacement, it is unclear whether a concomitant aortic valve replacement should be performed. Methods: From June 2002 to January 2020, 75 patients with mild-to-moderate BAV regurgitation (± mild-to-moderate stenosis) who underwent isolated supracoronary ascending aorta replacement were retrospectively analyze. Clinical and echocardiographic follow-up was 100% complete (mean: 7.4±3.9 years, max 16.4). Kaplan Meier estimates were employed to analyze long-term survival. Cumulative incidence function for time to re-operation, recurrence of aortic regurgitation (AR)≥3+ and aortic stenosis (AS) greater than moderate, with death as competing risk, were computed. Results: There was no hospital mortality and no cardiac death occurred. Overall survival at 12 years was 97.4±2.5%, 95% CI [83.16-99.63]. At follow-up there were no cases of aortic root surgery whereas 3 patients underwent AV replacement. At 12 years the CIF of reoperation was 2.6±2.5%, 95% CI [0.20-11.53]. At follow up, AR 3+/4+ was present in 1 pt and AS greater than moderate in 3. At 12 years the CIF of AR>2+/4+ was 5.1±4.98% and of AS>moderate 6.9±3.8%. Conclusions: In our study mild to moderate regurgitation of a BAV did not significantly worse at least up to 10 years after isolated supracoronary ascending aorta replacement.


2020 ◽  
Vol 47 (1) ◽  
pp. 15-22
Author(s):  
Kai Ma ◽  
Lei Qi ◽  
Zhongdong Hua ◽  
Keming Yang ◽  
Hao Zhang ◽  
...  

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied by left ventricular outflow tract obstruction and ventricular septal defect and who had then undergone palliative bidirectional Glenn shunt placement at our institution from January 2005 through December 2014. Patients were divided into 3 groups according to subsequent surgeries: Fontan completion (total cavopulmonary connection, 13 patients) (group 1), anatomic repair (hemi-Mustard and Rastelli procedures without Glenn takedown, 11 patients) (group 2), and prolonged palliation (no further surgery, 26 patients) (group 3). After shunt placement, no patient died or had ventricular dysfunction. Overall, mean oxygen saturation increased significantly from 79.5% ± 13.5% preoperatively to 94.1% ± 7.3% (P <0.001). The median time from shunt placement to Fontan completion and anatomic repair, respectively, was 2.1 years (range, 1.6–5.2 yr) and 1.1 years (range, 0.6–2.4 yr). Only 2 late deaths occurred, both in group 1. In group 3, time from shunt placement to latest follow-up was 4.5 years (range, 2.3–8 yr). At latest follow-up, mean oxygen saturation was 91.6% ± 10.3%, and no patients had impaired ventricular function. Bidirectional Glenn shunt placement as an optional palliative procedure for complex congenitally corrected transposition has favorable outcomes. Later, patients can feasibly be treated by Fontan completion or anatomic repair. Use of a bidirectional Glenn shunt for open-ended palliation is also acceptable.


2020 ◽  
Vol 26 (4) ◽  
pp. 4-12
Author(s):  
A.А. Malska ◽  
◽  
O.B. Kuryliak ◽  

Aim. To determine the rate of aortic coarctation, the correlation of its anatomical forms - critical and not critical, and the frequency of combination with the associated pathology; to define the features of the clinical course of its different anatomical forms; and to analyze the remote results of the surgical correction of this defect. Material and Methods. The article represents the statistical analysis of outpatient medical records and case histories of 86 children with aortic coarctation in Lviv region. In the course of the research, retrospective and epidemiological studies were carried out; clinical (data acquisition of medical history, physical examination), instrumental (Doppler echocardiography, ECG, X-ray imaging of organs of the thoracic cavity), and statistical methods were used. Results and Discussion. It was determined that over the period of 2008-2020 years, out of 74 neonates with CoA registered at Lviv Regional Children's Hospital (Health Care of Mother and Child) 40,54% had the critical CoA form, while 59 (46%) - uncritical CoA form. In children with the critical CoA form, the most frequent findings were hypoplasia of the aortic arch (56,67%), open aortic duct (53,33%), and open oval window (53,33%); in 36,6% cases CoA was combined with the bicuspid aortic valve, interatrial septal defect, and transposition of great vessels. However, the uncritical CoA form was more frequently combined with the bicuspid aortic valve (52,27%), and hypoplastic aortic arch (31,82%); aortic stenosis was revealed in 20,45% of children. After the surgical correction in 43,59% of the operated patients with uncritical CoA, excessive arterial hypertension was observed, while in critical form, the frequency of excessive arterial hypertension among the operated patients amounted to 10%. According to our research, after the plasty of the critical CoA, aortic recoarctation was observed in 3.33% of the operated patients, whereas, in case of the uncritical CoA form, it occurred in 30,77% of the operated ones. Conclusions. Coarctation of the aorta is a congenital heart disease with relatively high incidence, amounting to 5-8% out of all congenital defects of the heart. In newborns, it is manifested by acute cardiac failure, while in elder children it is presented with arterial hypertension. Echocardiographic examination after Doppler analysis is the basic procedure of diagnostics and allows for precise determination of the CoA anatomy. The majority of cardiologists recommend prompt surgical intervention after the diagnosis has been made, and, particularly, in patients with hypertension. At present, the available surgical methods include surgical excision of the aortic obstruction, and catheter intervention (the balloon angioplasty and stent implantation) . After the surgical correction has been performed, arterial hypertension persists. The duration of hypertension after the coarctation correction depends on its duration before the diagnosis is made and the timing of surgical correction of the defect. With the child's growth , recoarctation may occur. In such patients, normal arterial pressure can be determined in the state of rest, but it may increase in the upper extremities during physical exertion. Key words: aortic coarctation, associated pathology, excessive arterial hypertension, recoarctation


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Rebecca R Hartog ◽  
Kimberly J Watkins ◽  
Megan Wilde ◽  
Tiffany R Lim ◽  
Andrew Rodenbarger ◽  
...  

Introduction: Limited data exist on the electrophysiologic outcomes of patients undergoing anatomic repair (AR) for congenitally corrected transposition of the great arteries (ccTGA). AR was defined as an atrial switch procedure plus either arterial switch (ASO) or Rastelli operation. Aims: To report mid and late electrophysiologic outcomes after AR and identify risk factors for those outcomes. Methods: Single center retrospective cohort study of patients undergoing AR between 1993-2017. Data were collected from available records. Transplant-free survival to 1 year post repair was required for inclusion. Standard descriptive statistical analysis and Cox proportional hazards were used. Results: Of 85 patients included, 95% had lesions in addition to ccTGA: most commonly VSD (84%) and pulmonary stenosis or atresia (58%). Median age at AR was 1.5y (IQR 0.9-2.8) with Senning/ASO in 56%, Senning/Rastelli in 38%, and hemi-Senning/Glenn/Rastelli in 6%. During a median follow-up of 10.6y, 45 (53%) patients developed an arrhythmia requiring intervention. Atrial tachycardia (AT) in 27 (32%) or ventricular tachycardia (VT) in 11 (13%) patients required intervention at a median of 7.4y (IQR 1.6-15.3y) and 15.9y (IQR 4.5-17.9) post-AR, respectively. Treatments included chronic medications in 29 (64%), cardioversion in 15 (33%) and catheter ablation in 10 (22%). Median freedom from AT and VT was 17.3y and 25y post-AR, respectively. D-looped ventricles (p=0.03) and multiple operations prior to AR (p=0.02) were associated with increased AT risk; and native pulmonary stenosis with increased VT risk (p=0.01). Those needing heart failure/transplant referral had increased risk of both AT and VT (both p=0.04). Pacemaker was implanted for heart block and/or SND prior to or during AR in 14 (16%), immediately post-op in 9 (11%), and late (median 6y post-AR) in 24 (28%). ICDs were implanted in 5 (6% of cohort), 4 for primary prevention. No patient had an appropriate shock. Conclusions: Anatomic ccTGA repair is associated with significant electrophysiologic morbidity. AT, VT, and SND develop at a similar incidence to that reported for d-TGA patients after atrial switch. The incidence of AV block follows a similar trajectory to that of physiologically palliated ccTGA.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Alexandra van Dissel ◽  
Alexander Opotowsky ◽  
Jamil A Aboulhosn ◽  
Martijn Kauling ◽  
Salil Ginde ◽  
...  

Background: Occasionally patients with congenitally corrected transposition of the great arteries (ccTGA) exhibit little clinical evidence of cardiovascular limitation even to their 8th decade. We aimed to assess survival prospects in a large cohort of ccTGA adults. Methods & Results: We included 555 ccTGA adults (median age 33.0 years, 48.3% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 8.1 [IQR 4.4 - 13.3] years, 56 (10.1%) patients died, 10 (1.8%) patients underwent MCS and 14 (2.5%) had a heart transplant. Median age at time of primary outcome was 51.1 [IQR 37.5 - 63.2] years and cumulative incidence at 15 years from baseline was 21.5% [95% CI 16.1 - 26.5]. Leading causes of death were worsening of heart failure (43%) and sudden death (10%). Patients who died were more likely to use heart failure (HF) medications. In multivariable Cox analyses for baseline variables, age, prior atrial arrhythmia and HF admission were each associated with an increased risk of the primary outcome. Figure shows cumulative incidence according to history of atrial arrhythmia. During follow-up, 91 (16.4%) were admitted for HF, pacemaker implantation was performed in 68 (12.3%) patients, ICD in 82 (14.7%), and major cardiac surgery (mostly for systemic AV-valve) in 89 (15.8%) patients. Conclusion: In this large cohort of ccTGA adults, survival seemed to be primarily determined by heart failure-related complications. Prior atrial arrhythmia also seems to be a harbinger for adverse outcome. Few patients underwent advanced HF therapies. Figure: Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 14 years from first visit at an adult congenital heart disease clinic since 2002 stratified according to history of atrial arrhythmia. Shading represents upper and lower 95% confidence limits.


ESC CardioMed ◽  
2018 ◽  
pp. 2861-2863
Author(s):  
Bernard Iung

Aortic diseases encountered in young women are mainly associated with syndromic diseases, which are often heritable, or bicuspid aortic valve. The most frequent syndromic disease is Marfan syndrome. In Marfan syndrome, the risk of aortic dissection is low during pregnancy when the maximum diameter of the ascending aorta is less than 45 mm. Dissection may affect the thoracic ascending or descending aorta. The risk of aortic dissection is low in bicuspid aortic valve when the aortic diameter is less than 50 mm. Beta blockers are recommended throughout pregnancy in Marfan syndrome and are often used in other causes of aortic aneurysms. Close echocardiographic follow-up is needed during pregnancy and after delivery.


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