A Rare Combination of Congenitally Corrected Transposition of the Great Arteries with Pulmonary Artery Aneurysm.
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ABSTRACT:
SUBMITTER: Kosmas N
PROVIDER: S-EPMC6200690 | biostudies-other | 2018 Oct
REPOSITORIES: biostudies-other
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Project description:We report a man with congenitally corrected transposition of the great arteries and moderate anteroseptal ischemia; and cardiac computed tomography showed a single coronary artery with origin from the right aortic sinus. The perfusion of the morphological right ventricle by a single right coronary artery may be a cause of ischemia. (Level of Difficulty: Beginner.).
| S-EPMC8310992 | biostudies-literature
Project description:Background:Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease which may present with sudden death from malignant arrhythmias including complete heart block and ventricular tachyarrhythmias as late complications. Only few cases about ventricular tachyarrhythmias, usually in those with markedly depressed systemic ventricular function, have been reported. Case summary:A 26-year-old woman with a known history of CCTGA presented to the emergency department with palpitations and breathlessness for 3-4?weeks and worsening symptoms for 8?h. She had a history of ventricular septal defect repair 14?years ago. Her initial presentation electrocardiogram demonstrated high degree atrioventricular block with a ventricular rate of 44 b.p.m. She had two episodes of complete syncope during this hospitalization, both required external defibrillation due to documented bradycardia-dependent ventricular fibrillations. Her two-dimensional echocardiography study confirmed the diagnosis of CCTGA with preserved systolic ventricular function. She underwent urgent temporal pacing wire placement with a paced ventricular rate at 90 b.p.m. Having thoroughly reviewed the arrhythmia events and discussed with the patient about the option of defibrillator vs. pacemaker therapy a decision was made upon her request for dual-chamber pacemaker implantation. She was discharged home uneventfully 3?days after hospital presentation and has been physically active at 3-, 6-, and 9-month follow-ups. Discussion:Our case illustrates the individualized clinical decision making in choosing device therapy for a rare congenital heart disease presented with malignant arrhythmia. Careful history taking, open communication, and closely planned long-term follow-up will be essential in caring for such patients.
| S-EPMC7042131 | biostudies-literature
Project description: Not available
| S-EPMC4665295 | biostudies-literature
Project description:We present a diagnosis of isolated anatomically corrected malposed great arteries on fetal echocardiography at 31 weeks of gestation period. The patient was referred to our institute with a diagnosis of suspected transposition of great arteries.
| S-EPMC5007939 | biostudies-literature
Project description:BackgroundPatients with congenitally corrected transposition of the great arteries (ccTGA) often develop tricuspid valve (systemic atrioventricular valve) dysfunction due to right ventricular overload and dilatation, but isolated mitral valve (MV) disease is rarely found. Isolated mitral (subpulmonic atrioventricular valve) interventions, specifically catheter-directed, have not been reported up to date.Case summaryA man with ccTGA is evaluated for dyspnoea. Multimodality imaging assessment confirmed severe right-sided MV regurgitation due to prolapse. In light of high surgical risk, a minimally invasive transcatheter MitraClip procedure was pursued.DiscussionTo our knowledge, this is the first case of successful MV repair via percutaneous approach using MitraClip in a patient with ccTGA and biventricular failure. Our case illustrates the safety and feasibility of the edge-to-edge procedure in such a rare instance, but also the importance of multimodality imaging (both invasive and non-invasive) and the Heart Team approach when caring for these complex patients.
| S-EPMC8759502 | biostudies-literature
Project description:BackgroundPatients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored.Case summaryA 38-year-old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, and no significant atrio-ventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit, and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Post-operative course was uneventful, transthoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At 1 month after discharge, the patient is in New York Heart Association Class II.DiscussionManagement of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with an anatomic repair.
| S-EPMC8759476 | biostudies-literature
Project description:BackgroundAdults with unoperated congenitally corrected transposition of the great arteries are rare but form a distinct group among adults with congenital heart disease. Patients with congenitally corrected transposition of the great arteries often have one or more associated cardiac anomalies that dictate the need for, and timing of, surgical intervention in childhood. However, in a proportion of patients, the hemodynamics does not require surgical attention during childhood, and, in some patients, a correct diagnosis is not established until adulthood. Here we report an adult case of unoperated congenitally corrected transposition of the great arteries with a large ventricular septal defect and probable pulmonary arterial hypertension.Case presentationOur patient was a 46-year-old Korean man. Transthoracic echocardiography and cardiac catheterization demonstrated hemodynamically balanced ventricles with a non-regurgitant systemic atrioventricular valve, normal pulmonary arterial pressure, and a reasonable difference between the oxygen saturation values of the aorta and pulmonary trunk, even with the presence of a large ventricular septal defect. Further morphological assessments using cardiac computed tomography and three-dimensional modeling/printing of his heart revealed that the mitral valve was straddling over the posteriorly positioned ventricular septal defect, which could explain the functional and anatomical subvalvular pulmonary stenosis and a small amount of shunt flow through the large ventricular septal defect. We interpreted this combination of cardiac defects as able to sustain his stable cardiac function. Thus, we decided to maintain his unoperated status.ConclusionA detailed anatomical understanding based on transthoracic echocardiography, cardiac computed tomography, and three-dimensional printing can justify a decision to not operate in cases of congenitally corrected transposition of the great arteries with hemodynamically balanced pulmonary stenosis and a ventricular septal defect, as observed in the present case.
| S-EPMC6639941 | biostudies-literature
Project description:Background:Limited research has been conducted on the surgical management of the aortic valve in congenitally corrected transposition of great arteries (ccTGA) and to our knowledge there have been no reports on the treatment of bicuspid aortic regurgitation (AR) in ccTGA. We report on a ccTGA patient with bicuspid AR and systemic right ventricule (SRV) dysfunction who underwent transcatheter aortic valve replacement (TAVR). Case summary:A 17-year-old male with a history of ccTGA and cerebral palsy diagnosed at birth presented with heart failure. During childhood, he did not experience any heart failure symptoms, however, secondary to progressive bicuspid AR he experienced worsening SRV dysfunction beginning at 15-year-old. Echocardiography showed reduced SRV ejection fraction and severe bicuspid AR. The heart team, including a cardiac surgeon and paediatric cardiologist, discussed the treatment strategies and decided to proceed with TAVR as surgical aortic valve replacement was deemed high risk. TAVR was performed with the 34?mm Evolut R (Medtronic, Minneapolis, MN, USA). Post-operative echocardiography showed severe paravalvular leak (PVL). Therefore, valve-in-valve TAVR using a 29?mm Edwards SAPIEN 3 (Edwards Lifesciences, Irvine, CA, USA) was performed on post-operative Day 2 for PVL reduction. Following second procedure, PVL was significantly improved. The patient was discharged in stable condition. Discussion:This is the first case wherein TAVR was performed for bicuspid AR in a patient with ccTGA. With appropriate preparation and planning and a collaborative multi-disciplinary team approach, TAVR can be a treatment option for severe AR in patients with ccTGA at high risk for surgery.
| S-EPMC7319830 | biostudies-literature
Project description:BackgroundPatients with congenitally corrected transposition of the great arteries (ccTGA) are prone to the development of advanced atrio-ventricular block requiring chronic ventricular pacing. The morphological right ventricle (RV) often develops systolic dysfunction as it is unable to withstand the chronic pressure overload it is exposed to when supporting the systemic circulation.Case summaryA 56-year-old woman with dextrocardia and complex ccTGA with a history of dual-chamber implantable cardioverter-defibrillator (DDD-ICD, high degree atrio-ventricular-block and syncopal ventricular tachycardia), presented with progressive heart failure and symptomatic atrial arrhythmias. She underwent a successful ablation and concomitant invasive haemodynamic evaluation of potential alternative/biventricular pacing modalities. During biventricular pacing, the QRS narrowed and the systemic RV intraventricular pressure (Dp/Dt) increased with 30%. She underwent a successful transvenous upgrade to cardiac resynchronization therapy (CRT). The electrocardiogram post-implantation showed biventricular capture and patient showed subjective and objective clinical improvement.DiscussionSystemic RV dysfunction in ccTGA can be aggravated by chronic pacing-induced dyssynchrony, contributing to progression of heart failure in this patient group. Transvenous CRT is feasible in ccTGA anatomy and may be pursued in order to improve or preserve the functional status of pacing-dependent ccTGA patients. Invasive haemodynamic contractility evaluation can help assess the potential benefit of CRT in patients with complex anatomy.
| S-EPMC8188871 | biostudies-literature
Project description:Several criteria to identify suitable candidates for anatomic repair in congenitally corrected transposition (cc-TGA) have been proposed. The purpose of this study was to critically re-evaluate adequacy of these recommendations in our patient cohort. All cc-TGA patients undergoing anatomic repair between 2010 and 2019 were reviewed. Evaluated eligibility criteria for repair included age ≤ 15 years, LV mass index ≥ 45-50 g/m2, LV mass/volume ratio > 0.9-1.5 and systolic LV to right ventricle pressure ratio > 70-90% among others. Repair failure was defined as postoperative early mortality or LV dysfunction requiring mechanical circulatory support. Twenty-five patients were included (median [interquartile range] age at surgery 1.8 years [0.7;6.6]; median postoperative follow-up 3.2 years [0.7;6.3]). Median preoperative LV ejection fraction was 60% [56;64], indexed LV mass 48.5 g/m2 [43.7;58.1] and LV mass/volume ratio 1.5 [1.1;1.6], respectively. A total of 12 patients (48%) did not meet at least one of the previously recommended criteria, however, all but two patients (92%) experienced favorable early outcome. Of 7 patients (28%) with indexed LV mass < 45 g/m2, 6 were successfully operated. There were two early repair failures (8%) with LV dysfunction: one patient died and one required mechanical circulatory support but recovered well. Surgery was performed successfully in patients with LV mass and volume Z-scores as low as - 2 and - 2.5, respectively. Anatomic correction for cc-TGA can be performed with excellent early outcome and is feasible even in patients with LV mass below previously recommended cut-offs. The use of LV mass and volume Z-scores might help to refine eligibility criteria.
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