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1.
J Cardiothorac Surg ; 19(1): 448, 2024 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-39004754

RESUMO

BACKGROUND: Extra-anatomic ascending-to-descending aortic bypass grafts have historically been utilized as a safe and effective solution for repairs of complex coarctation of the aorta. However, reports on reoperation in these patients remain rare. We present a case of an aortic valve replacement and coronary artery bypass grafting in a patient with an extra-anatomic ascending-to-descending aortic bypass graft. CASE PRESENTATION: The patient is a 59-year-old male with a complex aortic history, including repair of aortic coarctation with an ascending-to-descending aortic bypass graft 13 years prior, was admitted to the hospital for shortness of breath and chest pain that had developed over the past year. On further workup, he was found to have severe bileaflet aortic valve stenosis, non-ST elevation myocardial infarction, and moderate coronary artery disease. He underwent surgical aortic valve replacement and coronary artery bypass grafting. Given his unique anatomy, cardiopulmonary bypass approach involved separate cannulation of the right axillary and left common femoral arteries with cross-clamp of both the aorta and the extra-anatomic graft. Using this approach, the redo operation was successfully performed. CONCLUSIONS: Reports on reoperation after ascending-to-descending aortic bypass grafting are rare. We describe our approach to cardiopulmonary bypass and reoperation in a patient with an extra-anatomic ascending-to-descending aortic bypass graft.


Assuntos
Ponte de Artéria Coronária , Reoperação , Humanos , Masculino , Pessoa de Meia-Idade , Ponte de Artéria Coronária/métodos , Implante de Prótese de Valva Cardíaca/métodos , Coartação Aórtica/cirurgia , Aorta/cirurgia , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia
3.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 49(4): 595-602, 2024 Apr 28.
Artigo em Inglês, Chinês | MEDLINE | ID: mdl-39019788

RESUMO

OBJECTIVES: Interruption of aortic arch (IAA) is a rare congenital heart disease. This study aims to investigate echocardiographic features and pathological ultrastructural characteristics of fetal IAA and to further analyze its pathological evolution. METHODS: A retrospective analysis was conducted on prenatal echocardiographic, post-surgical, or autopsy findings of fetuses prenatally diagnosed with IAA. Prenatal echocardiographic tracking was used to observe the internal diameters and Z-scores of different segments of the aortic arch and the changes in the narrowed section. These observations were combined with autopsy and pathological findings to explore the potential intrauterine evolution of IAA and its cytological basis. RESULTS: The study included 34 fetuses with IAA, with 3, 3, and 28 fetuses prenatally diagnosed with aortic arch dysplasia (AAD), coarctation of aorta (CoA), and IAA, respectively. The 3 AAD and 3 CoA fetuses chose termination of pregnancy 1 to 2 weeks after prenatal ultrasound diagnosis, and autopsy confirmed IAA. Among the 28 fetuses prenatally diagnosed with IAA, 6 cases of CoA progressively worsened, eventually evolving into type A IAA as observed through echocardiographic follow-up. The remaining 22 cases were diagnosed as IAA on the first prenatal ultrasound. Postnatal surgery corrected 3 cases, while 27 cases opted for pregnancy termination, and 4 cases resulted in intrauterine death. Echocardiographic features of the fetal IAA included a significantly smaller left ventricle compared with the right or negligible difference on the four-chamber view, a significantly smaller aorta than the pulmonary artery on the three-vessel view, and a lack of connection between the aorta and the descending aorta on the three-vessel-trachea and aortic arch views. The aortic arch appears less curved and more rigid, losing the normal "V" shape between the aorta, ductus arteriosus, and descending aorta. Color Doppler ultrasound showed no continuous blood flow signal at the interruption site, with reversed blood flow visible in the ductus arteriosus. Transmission electron microscopy of 7 IAA fetuses revealed numerous disorganized smooth muscle cells between the elastic membranes near the aortic arch interruption site, significantly increased in number compared with the proximal ascending aorta. The elastic membranes were thicker and more twisted near the interruption site. The interruption area lacked normal endothelial cells and lumen, with only remnants of necrotic endothelial cells, disorganized short and thick elastic membranes, and randomly arranged smooth muscle cells. CONCLUSIONS: Prenatal echocardiography is the primary diagnostic tool for fetal IAA. Post-surgical follow-up and autopsy help identify complications and disease characteristics, enhancing diagnostic accuracy. Some fetal IAA may evolve from AAD or CoA, with potential pathogenesis related to ischemia, hypoxia, and migration of ductal constrictive components.


Assuntos
Aorta Torácica , Ultrassonografia Pré-Natal , Humanos , Feminino , Aorta Torácica/embriologia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/patologia , Gravidez , Estudos Retrospectivos , Ecocardiografia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/embriologia , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/patologia , Coartação Aórtica/embriologia , Adulto
4.
Birth Defects Res ; 116(7): e2382, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38975735

RESUMO

BACKGROUND: A peculiar subgroup of patients with partial or complete atrioventricular canal defect exhibits a spectrum of left-sided obstructions including right ventricular dominance and aortic coarctation. The association of atrioventricular canal defect with left-sided obstructions is found in several genetic syndromes; however, the molecular basis of nonsyndromic atrioventricular canal defect with aortic coarctation is still poorly understood. Although some candidate genes for nonsyndromic atrioventricular canal defect are known, a complex oligogenic inheritance determined in some cases by the co-occurrence of multiple variants has also been hypothesized. CASE REPORT: We describe a nonsyndromic infant with mesocardia with viscero-atrial situs solitus, partial atrioventricular canal defect, mild right ventricular dominance, and coarctation of the aorta. Next generation sequencing genetic testing revealed variants in two genes, GDF1 and NOTCH1, previously reported in association with atrioventricular canal defect and left-sided obstructive lesions, respectively. CONCLUSION: The present report could support the hypothesis that the co-occurrence of cumulative variants may be considered as genetic predisposing risk factor for specific congenital heart defects.


Assuntos
Coartação Aórtica , Fator 1 de Diferenciação de Crescimento , Receptor Notch1 , Humanos , Coartação Aórtica/genética , Receptor Notch1/genética , Fator 1 de Diferenciação de Crescimento/genética , Defeitos dos Septos Cardíacos/genética , Masculino , Feminino , Predisposição Genética para Doença , Recém-Nascido , Mutação/genética , Lactente
5.
BMJ Case Rep ; 17(6)2024 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-38901852

RESUMO

Mid-aortic syndrome (MAS) is a rare vascular disease that usually leads to renovascular hypertension. With the predominant manifestations being intractable arterial hypertension and lower extremity arterial insufficiency, it has rarely been associated with dilated cardiomyopathy. We report a young girl with congestive heart failure, where the cause was initially attributed to dilated cardiomyopathy. A repeated echocardiogram 6 months later brought the physician's suspicion of MAS because of the abnormal colour of Doppler from the subcostal view. Further assessment using CT angiography revealed discrete thoracic coarctation at the level of T10, with the narrowest diameter of 2.1 mm, thus confirming the diagnosis. Her inflammatory markers and connective tissue screening were negative. She underwent successful stenting of coarctation of the aorta, which later caused improvement in her cardiac function. We highlighted the importance of looking for treatable causes of dilated cardiomyopathy and vigilant clinical and echocardiogram assessment with high suspicion to diagnose MAS.


Assuntos
Coartação Aórtica , Cardiomiopatia Dilatada , Humanos , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/complicações , Feminino , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico , Coartação Aórtica/diagnóstico por imagem , Angiografia por Tomografia Computadorizada , Ecocardiografia , Stents , Diagnóstico Diferencial , Síndrome , Insuficiência Cardíaca/etiologia
6.
Radiol Cardiothorac Imaging ; 6(3): e230403, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38900025

RESUMO

Generalized arterial calcification of infancy (GACI) is a rare genetic condition with varied clinical presentation. Consequently, diagnosis is frequently delayed or missed. GACI has a poor prognosis, with more than half of patients dying before the age of 6 months. Early diagnosis and treatment with bisphosphonates have been shown to improve survival in these patients. This is a case report of a newborn with respiratory distress who was initially diagnosed with coarctation of the aorta at echocardiography. Further imaging with CT revealed the aortic narrowing to be associated with GACI. Keywords: Genetic Defects, Congenital, Vascular, Calcification/Calculi, Aorta, Pulmonary Arteries, CT Angiography, Echocardiography, Pediatrics © RSNA, 2024.


Assuntos
Coartação Aórtica , Ecocardiografia , Calcificação Vascular , Humanos , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/diagnóstico , Recém-Nascido , Calcificação Vascular/diagnóstico por imagem , Calcificação Vascular/diagnóstico , Diagnóstico Diferencial , Masculino , Angiografia por Tomografia Computadorizada , Feminino
7.
J Cardiothorac Surg ; 19(1): 354, 2024 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-38909233

RESUMO

BACKGROUND: A left thoracotomy approach is anatomically appropriate for childhood aortic coarctation; however, the pediatric femoral arteriovenous diameters are too small for cardiopulmonary bypass cannulation. We aimed to determine the safety of a partial cardiopulmonary bypass through the main pulmonary artery and the descending aorta in pediatric aortic coarctation repair. METHODS: We retrospectively reviewed 10 patients who underwent coarctation repair under partial main pulmonary artery-to-descending aorta cardiopulmonary bypass with a left thoracotomy as the CPB group. During the same period, 16 cases of simple coarctation of the aorta repair, with end-to-end anastomosis through a left thoracotomy without partial CPB assistance, were included as the non-CPB group to evaluate the impact of partial CPB. RESULTS: The median age and weight at surgery of the CPB group were 3.1 years (range, 9 days to 17.9 years) and 14.0 (range, 2.8-40.7) kg, respectively. Indications for the partial cardiopulmonary bypass with overlap were as follows: age > 1 year (n = 7), mild aortic coarctation (n = 3), and predicted ischemic time > 30 min (n = 5). Coarctation repair using autologous tissue was performed in seven cases and graft replacement in three. The mean partial cardiopulmonary bypass time, descending aortic clamp time, and cardiopulmonary bypass flow rate were 73 ± 37 min, 57 ± 27 min, and 1.6 ± 0.2 L/min/m2, respectively. Urine output during descending aortic clamping was observed in most cases in the CPB group (mean: 9.1 ± 7.9 mL/kg/h), and the total intraoperative urine output was 3.2 ± 2.7 mL/kg/h and 1.2 ± 1.5 mL/kg/h in the CPB and non-CPB group, respectively (p = 0.020). The median ventilation time was 1 day (range, 0-15), and the intensive care unit stay duration was 4 days (range, 1-16) with no surgical deaths. No major complications, including paraplegia or recurrent coarctation, occurred postoperatively during a median observation period of 8.1 (range, 3.4-17.5) years in the CPB group. In contrast, reoperation with recurrent coarctation was observed in 2 cases in the non-CPB group (p = 0.37). CONCLUSIONS: Partial cardiopulmonary bypass through the main pulmonary artery and descending aorta via a left thoracotomy is a safe and useful option for aortic coarctation repair in children.


Assuntos
Coartação Aórtica , Ponte Cardiopulmonar , Toracotomia , Humanos , Coartação Aórtica/cirurgia , Estudos Retrospectivos , Ponte Cardiopulmonar/métodos , Pré-Escolar , Criança , Lactente , Toracotomia/métodos , Masculino , Feminino , Adolescente , Recém-Nascido , Aorta Torácica/cirurgia , Artéria Pulmonar/cirurgia , Resultado do Tratamento
8.
Medicina (Kaunas) ; 60(6)2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38929503

RESUMO

Objectives: The configuration of the aortic arch, particularly a Gothic arch shape, in individuals with corrected coarctation of the aorta (CoA) has been associated with a decreased systolic wave amplitude across the arch, which could potentially impair renal perfusion and elevate the risk of arterial hypertension. This study aims to explore the relationship between the morphological characteristics of the aortic arch and their impact on renal perfusion in patients with CoA. Methods: Seventy-one subjects with corrected CoA underwent continuous 24 h ambulatory blood pressure monitoring, computed tomography to assess the aortic arch, and renal perfusion scanning. Subjects were stratified into three groups based on the height-to-width (H/W) ratio of their aortic arch: Group 1 with a H/W ratio of <0.65, Group 2 with a H/W ratio between 0.65 and 0.85, and Group 3 with a H/W ratio of >0.85. Results: Groups 1 and 2 (53,78% and 62.63%) presented with a higher hypertension prevalence of elevated blood pressure than Group 3 (38.89%). Notable variations were observed among the subjects in the time to peak perfusion (Tmax) in the left kidney across the groups. Group 1 showed a median Tmax at 0.27, Group 2 at 0.13, and Group 3 at -0.38 (p-value = 0.079). The differences in Tmax for the right kidney followed a similar trend but were not statistically significant (Group 1 at 0.61, Group 2 at 0.22, and Group 3 at 0.11; p-value = 0.229). Conclusions: This study suggests that variations in the aortic arch morphology might not significantly influence renal perfusion in CoA patients. This indicates the potential adaptability of the renal blood flow, which appears to compensate for reduced perfusion, thus minimizing adverse effects on the kidney function. This adaptability suggests an inherent physiological resilience, emphasizing the need for further targeted research to understand the specific interactions and impacts on treatment strategies for CoA.


Assuntos
Aorta Torácica , Coartação Aórtica , Humanos , Coartação Aórtica/fisiopatologia , Coartação Aórtica/diagnóstico por imagem , Feminino , Masculino , Adulto , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/fisiopatologia , Hipertensão/fisiopatologia , Hipertensão/complicações , Rim/fisiopatologia , Rim/irrigação sanguínea , Rim/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Monitorização Ambulatorial da Pressão Arterial/métodos , Pessoa de Meia-Idade , Adolescente
9.
BMC Cardiovasc Disord ; 24(1): 330, 2024 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-38943106

RESUMO

BACKGROUND: The purpose of this study was to review echocardiography-based diagnosis of persistent fifth aortic arch (PFAA) in children. METHODS: From January 2015 to December 2022, we retrospectively analyzed the echocardiographic findings and the relevant clinical data during follow-up of patients with PFAA who were treated in the Third Affiliated Hospital of Zhengzhou University. The diagnosis was confirmed by computed tomography angiography or surgery. RESULTS: Seven PFAA cases included two Weinberg type A and five Weinberg type B. The anatomical details of PFAA were assessed using a combination of the long-axis view of the left ventricular outflow tract (from the left high parasternal window) and the long-axis view of the aortic arch (from the suprasternal window). In Weinberg type A, the distal fifth and fourth aortic arches were connected to the descending aorta, which was associated with aortic coarctation. In Weinberg type B, the upper arch of the fourth aorta was interrupted, and only the lower arch of the fifth aorta was connected to the descending aorta. Surgical repair of PFAA was indicated in five patients with blood flow disruption, among which four had good postoperative results and one refused surgery. Two patients with unobstructed PFAA blood flow required follow-up rather than surgery. CONCLUSIONS: It is feasible to diagnose PFAA by echocardiography. Combined application of the high parasternal left ventricular outflow tract view and the suprasternal aortic arch view can improve timely detection of different types of PFAA in children.


Assuntos
Aorta Torácica , Angiografia por Tomografia Computadorizada , Valor Preditivo dos Testes , Humanos , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Estudos Retrospectivos , Masculino , Feminino , Lactente , Pré-Escolar , Aortografia , Criança , China , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgia , Resultado do Tratamento , Fatores Etários , Reprodutibilidade dos Testes , Ecocardiografia
11.
Open Heart ; 11(1)2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38806222

RESUMO

OBJECTIVE: This study aims to compare aortic morphology between repaired coarctation patients and controls, and to identify aortic morphological risk factors for hypertension and cardiovascular events (CVEs) in coarctation patients. METHODS: Repaired coarctation patients with computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were included, followed-up and compared with sex-matched and age-matched controls. Three-dimensional aortic shape was reconstructed using patients' CTA or MRA, or four-dimensional flow cardiovascular magnetic resonance in controls, and advanced geometrical characteristics were calculated and visualised using statistical shape modelling. In patients, we examined the association of geometrical characteristics with (1) baseline hypertension, using multivariable logistic regression; and (2) cardiovascular events (CVE, composite of aortic complications, coronary artery disease, ventricular arrhythmias, heart failure hospitalisation, stroke, transient ischaemic attacks and cardiovascular death), using multivariable Cox regression. The least absolute shrinkage and selection operator (LASSO) method selected the most informative multivariable model. RESULTS: Sixty-five repaired coarctation patients (23 years (IQR 19-38)) were included, of which 44 (68%) patients were hypertensive at baseline. After a median follow-up of 8.7 years (IQR 4.8-15.4), 27 CVEs occurred in 20 patients. Aortic arch dimensions were smaller in patients compared with controls (diameter p<0.001, wall surface area p=0.026, volume p=0.007). Patients had more aortic arch torsion (p<0.001) and a higher curvature (p<0.001). No geometrical characteristics were associated with hypertension. LASSO selected left ventricular mass, male sex, tortuosity and age for the multivariable model. Left ventricular mass (p=0.014) was independently associated with CVE, and aortic tortuosity showed a trend towards significance (p=0.070). CONCLUSION: Repaired coarctation patients have a smaller aortic arch and a more tortuous course of the aorta compared with controls. Besides left ventricular mass index, geometrical features might be of importance in long-term risk assessment in coarctation patients.


Assuntos
Coartação Aórtica , Angiografia por Tomografia Computadorizada , Angiografia por Ressonância Magnética , Humanos , Coartação Aórtica/cirurgia , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico por imagem , Masculino , Feminino , Angiografia por Tomografia Computadorizada/métodos , Adulto , Fatores de Risco , Adulto Jovem , Seguimentos , Fatores de Tempo , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Estudos Retrospectivos , Imagem Cinética por Ressonância Magnética/métodos , Medição de Risco/métodos , Resultado do Tratamento , Hipertensão/complicações , Hipertensão/fisiopatologia , Adolescente
12.
Catheter Cardiovasc Interv ; 104(1): 61-70, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38736246

RESUMO

BACKGROUND: Stent implantation has become standard of care in older children and adults for treatment of branch pulmonary artery stenosis (BPAS) and coarctation aorta (CoAo). There are no stents approved or available for infants that have the potential to be dilated to adult diameters. The Minima stent was designed to fulfill this unmet need. METHODS: Multicenter, prospective, nonrandomized early feasibility study evaluating safety and effectiveness of the Minima stent for treatment of BPAS and CoAo. Primary endpoints included: (1) successful deployment across lesion, (2) stenosis relief defined by an increase in angiographic diameter of >50% and (3) freedom from stent explant, embolization or migration at 30 days and 6 months. RESULTS: Between 2/2022 and 5/2022, 10 pts underwent Minima stent implantation with a median age and weight of 9 months (4-43 months) and 7.6 kg (5.1-16.9 kg). Procedural success and predefined stenosis relief was achieved in all cases (CoAo [n = 4], BPAS [n = 6]). Adverse events occurred in 3 pts: transient diminished lower extremity pulse (n = 2), distal stent on-balloon displacement successfully managed in the catheterization suite (n = 1). There were no deaths or major adverse events. All patients were free from stent explant and migration at 30 days and 6 months with no evidence for significant restenosis at latest follow-up. CONCLUSIONS: Implantation of the Renata Minima stent was safe and effective for the treatment of BPAS and CoAo in this small cohort of infants and young children during early follow-up. Based on these early results, an expanded study with longer follow-up is warranted.


Assuntos
Coartação Aórtica , Estudos de Viabilidade , Desenho de Prótese , Estenose de Artéria Pulmonar , Stents , Humanos , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/terapia , Coartação Aórtica/fisiopatologia , Lactente , Estudos Prospectivos , Masculino , Feminino , Resultado do Tratamento , Estenose de Artéria Pulmonar/diagnóstico por imagem , Estenose de Artéria Pulmonar/fisiopatologia , Estenose de Artéria Pulmonar/terapia , Estenose de Artéria Pulmonar/etiologia , Fatores de Tempo , Pré-Escolar , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Fatores Etários , Angioplastia com Balão/instrumentação , Angioplastia com Balão/efeitos adversos
13.
Kardiol Pol ; 82(6): 625-631, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38767163

RESUMO

BACKGROUND: The exact prevalence and clinical significance of excessive increase in blood pressure in response to exercise in patients with repaired coarctation of aorta (CoA) remains unknown. AIM: This study aimed to investigate the impact of different definitions of exercise-induced hypertension (EIH) on the prevalence rates in our adult patients with repaired CoA. A systematic review of the available literature was also performed. METHODS: We retrospectively analyzed exercise test data from adult patients with repaired CoA followed at the national referral center for adult congenital heart disease between 1998 and 2021. The three most reported definitions of EIH in patients with repaired CoA were used for the analysis of EIH prevalence. We also performed a systematic search of the PubMed electronic bibliographic database. Full-text versions of all potentially relevant articles on EIH in CoA were reviewed for relevance. RESULTS: Our registry included 161 adult CoA patients. Complete exercise test results were available in 74 patients (59% male, median age 39 years [range 20-68 years]). The prevalence of EIH in our cohort varied from 24 to 41%, depending on the definition used. We identified eleven eligible articles from 184 publications. The reported prevalence rate of EIH in the studies ranged from 13% to 82%. CONCLUSION: EIH is common in patients with repaired CoA; however, the rates of EIH vary greatly depending on the definition used. A standardized and uniform EIH definition is needed to accurately assess the prevalence and clinical relevance of EIH in this population.


Assuntos
Coartação Aórtica , Exercício Físico , Hipertensão , Humanos , Coartação Aórtica/cirurgia , Adulto , Masculino , Hipertensão/etiologia , Hipertensão/epidemiologia , Feminino , Pessoa de Meia-Idade , Idoso , Estudos Retrospectivos , Adulto Jovem , Prevalência , Teste de Esforço
14.
BMC Pediatr ; 24(1): 317, 2024 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-38720245

RESUMO

BACKGROUND: Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS. METHODS: This study included TS patients admitted to Shenzhen Children's Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada's method). RESULTS: A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m2, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score. CONCLUSIONS: The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.


Assuntos
Síndrome de Turner , Humanos , Síndrome de Turner/complicações , Criança , Adolescente , Feminino , China/epidemiologia , Dilatação Patológica/etiologia , Masculino , Estudos Retrospectivos , Aorta/patologia , Aorta/diagnóstico por imagem , Coartação Aórtica , Doença da Válvula Aórtica Bicúspide/complicações , Pré-Escolar , Incidência , População do Leste Asiático
15.
Ann Card Anaesth ; 27(1): 82-84, 2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-38722130

RESUMO

ABSTRACT: Paraparesis following cardiac surgery is a manifestation of spinal cord injury (SCI). It can occur in any aortic surgery from the aneurysm to the coarctation of the aorta (CoA) where the cross-clamp of the aorta is applied. Though the incidence of paraplegia is low, its occurrence affects the morbidity and mortality of the patient. There are only sporadic case reports on the development of paraplegia following recurrent and technically challenging repair of CoA. However, the spontaneous development of paraplegia has also been reported in cases of unoperated CoA. The present report describes the case of delayed SCI in which paraparesis developed 5 days post a coarctation repair. The risk factors and strategies to protect the spinal cord during aortic surgeries are emphasized.


Assuntos
Coartação Aórtica , Paraparesia , Complicações Pós-Operatórias , Humanos , Coartação Aórtica/cirurgia , Paraparesia/etiologia , Complicações Pós-Operatórias/etiologia , Masculino , Traumatismos da Medula Espinal/complicações , Traumatismos da Medula Espinal/etiologia
16.
J Clin Pediatr Dent ; 48(3): 166-170, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38755995

RESUMO

PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as "ghost teeth", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child's dentition as it develops.


Assuntos
Coartação Aórtica , Anormalidades do Olho , Síndromes Neurocutâneas , Odontodisplasia , Humanos , Masculino , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico por imagem , Odontodisplasia/diagnóstico por imagem , Anormalidades do Olho/complicações , Pré-Escolar , Síndromes Neurocutâneas/complicações , Fossa Craniana Posterior/anormalidades , Fossa Craniana Posterior/diagnóstico por imagem , Extração Dentária
17.
Rev Assoc Med Bras (1992) ; 70(5): e20231626, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38775513

RESUMO

OBJECTIVE: This study aimed to compare the effectiveness of resection and extended end-to-end anastomosis between neonate and infant patients with coarctation. METHODS: This study was designed retrospectively and included 41 neonate (<30 days) and infant (30 days to 1 year) patients who were operated on using the resection and extended end-to-end anastomosis technique for aortic coarctation. Preoperative aortic annulus diameters and Z scores, all aortic arch diameters and Z scores, the presence of hypoplastic aortic segment, and the presence of prematurity were reviewed in both groups. Subsequently, we investigated whether these parameters were statistically related to the residual gradient in the operation area, whether there was a need for early re-intervention, and what was the incidence of mortality in the early postoperative period. In addition, the aortic arch Z scores of the patients at 6 months postoperatively were examined. RESULTS: While the mean age (p<0.001), body weight (p<0.001), and proximal arch Z score (p=0.029) were found to be significantly lower in the neonate group than in the infant group, the total length of the intensive care unit stay (p=0.013) and the total length of hospital stay (p=0.017) were found to be significantly higher. In addition, significant enlargement was detected in the proximal arch, distal arch, and isthmus segments in both patient groups. CONCLUSION: The resection and extended end-to-end anastomosis is an equally effective technique that can provide a marked decrease in gradient in the coarctation area and a significant enlargement of the aortic arch segments in the early period after coarctation repair in both neonate and infant patients.


Assuntos
Coartação Aórtica , Tempo de Internação , Humanos , Coartação Aórtica/cirurgia , Recém-Nascido , Lactente , Resultado do Tratamento , Estudos Retrospectivos , Feminino , Masculino , Anastomose Cirúrgica/métodos , Aorta Torácica/cirurgia , Fatores Etários
18.
Kyobu Geka ; 77(5): 345-350, 2024 May.
Artigo em Japonês | MEDLINE | ID: mdl-38720602

RESUMO

Aortic coarctation is diagnosed in approximately 5% of adult patients with congenital heart disease and is commonly diagnosed through the close examination of hypertension. Various surgical strategies for adult coarctation have been recently reported. Generally, aortic replacement may require blood transfusion in case of injury of the well-developed collateral vessels. Therefore, in order to secure an operative safety, we preoperatively used a medical image viewer to identify the abnormal vessels by three-dimensional computer graphics (3DCG) reconstruction. A 34-year-old male patient was referred to our hospital with hypertension and low ankle-brachial pressure index( ABI). Chest computed tomography( CT) scan showed aortic coarctation and development of abnormal collateral vessels. Descending aorta was replaced via a left third-fourth intercostal thoracotomy under partial extracorporeal circulation. As the image viewer depicted, anatomical abnormality of the collateral vessels was identified precisely, and surgically treated without any injury. The patient was discharged 10 days postoperatively without transfusion and with a normalized ABI.


Assuntos
Aorta Torácica , Coartação Aórtica , Imageamento Tridimensional , Humanos , Coartação Aórtica/cirurgia , Coartação Aórtica/diagnóstico por imagem , Masculino , Adulto , Aorta Torácica/cirurgia , Aorta Torácica/diagnóstico por imagem , Tomografia Computadorizada por Raios X
19.
Sci Rep ; 14(1): 11748, 2024 05 23.
Artigo em Inglês | MEDLINE | ID: mdl-38783056

RESUMO

The present study aimed to evaluate the outcomes of percutaneous treatment of aortic coarctation using self-expandable uncovered Nitinol stents. We conducted a retrospective clinical data review of all patients with aortic coarctation and treated with self-expandable uncovered Nitinol stents at our institution between 2009 and 2019. The gradient pressure across the coarctation site was measured using aortography. Follow-up echocardiography and computed tomography angiography were performed to assess possible stent complications. A total of 127 stents were successfully implanted in 125 patients (64.8% males) with a mean age of 35.36 ± 11.9 years. The gradient across the coarctation site decreased significantly from 67.48 ± 14.79 to 5.04 ± 3.01 mmHg (P < 0.001) after self-expandable stent implantation. Systolic blood pressure (SBP) decreased significantly from 175.53 ± 15.99 to 147.22 ± 12.83 mmHg (P < 0.001) after self-expandable stenting. There were no major technical or clinical complications, including balloon rupture, aneurysmal formation, infection, secondary stent migration, thrombosis, death during the procedure, and in-hospital mortality. On a mean follow-up of 48 ± 23.6 months (12-120 months), the gradient [from 59.43 ± 15.42 to 3.72 ± 1.38 mmHg (P < 0.001)] and SBP [from 175.53 ± 15.99 to 127.99 ± 7.82 mmHg (P < 0.001)] decreased significantly. There was no mortality, aneurysmal formation in the stent site, dislocation, or aortic re-stenosis requiring intervention during mid-term follow-up. Treatment of aortic coarctation using a self-expandable uncovered nitinol stent is safe and effective with promising mid-term outcomes.


Assuntos
Coartação Aórtica , Humanos , Coartação Aórtica/cirurgia , Coartação Aórtica/terapia , Masculino , Feminino , Adulto , Estudos Retrospectivos , Resultado do Tratamento , Pessoa de Meia-Idade , Stents Metálicos Autoexpansíveis/efeitos adversos , Ligas , Stents/efeitos adversos , Angiografia por Tomografia Computadorizada , Adulto Jovem , Seguimentos
20.
World J Pediatr Congenit Heart Surg ; 15(3): 332-339, 2024 05.
Artigo em Inglês | MEDLINE | ID: mdl-38646823

RESUMO

Objective: Hands-on surgical training (HOST) for congenital heart surgery (CHS), utilizing silicone-molded models created from 3D-printing of patients' imaging data, was shown to improve surgical skills. However, the impact of repetition and frequency of repetition in retaining skills has not been previously investigated. We aimed to longitudinally evaluate the outcome for HOST on two example procedures of different technical difficulties with repeated attempts over a 15-week period. Methods: Five CHS trainees were prospectively recruited. Repair of coarctation of the aorta (CoA) and arterial switch operation (ASO) were selected as example procedures of relatively low and high technical difficulty. Procedural time and technical performance (using procedure-specific assessment tools by the participant, a peer-reviewer, and the proctor) were measured. Results: Coarctation repair performance scores improved after the first repetition but remained unchanged at the follow-up session. Likewise, CoA procedural time showed an early reduction but then remained stable (mean [standard deviation]: 29[14] vs 25[15] vs 23[9] min at 0, 1, and 4 weeks). Conversely, ASO performance scores improved during the first repetitions, but decreased after a longer time delay (>9 weeks). Arterial switch operation procedural time showed modest improvements across simulations but significantly reduced from the first to the last attempt: 119[20] versus 106[28] min at 0 and 15 weeks, P = .049. Conclusions: Complex procedures require multiple HOST repetitions, without excessive time delay to maintain long-term skills improvement. Conversely, a single session may be planned for simple procedures to achieve satisfactory medium-term results. Importantly, a consistent reduction in procedural times was recorded, supporting increased surgical efficiency.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Competência Clínica , Humanos , Estudos Prospectivos , Procedimentos Cirúrgicos Cardíacos/métodos , Modelos Anatômicos , Cardiopatias Congênitas/cirurgia , Silicones , Impressão Tridimensional , Masculino , Feminino , Estudos Longitudinais , Coartação Aórtica/cirurgia , Educação de Pós-Graduação em Medicina/métodos
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