scholarly journals Disorder of Sexual Development and Congenital Heart Defect in 47XYY: Clinical Disorder or Coincidence?

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Hanane Latrech ◽  
Imane Skikar ◽  
Mohammed El Hassan Gharbi ◽  
Abdelmjid Chraïbi ◽  
Ahmed Gaouzi
Keyword(s):  
Coronary Artery ◽  
Pulmonary Artery ◽  
Physical Examination ◽  
Sexual Development ◽  
Congenital Heart ◽  
Left Coronary Artery ◽  
Left Ventricular ◽  
Tall Stature ◽  
First Case ◽  
Disorder Of Sexual Development

Background. 47XYY syndrome is a rare sex chromosome variation characterized by an additional Y chromosome. Most patients with 47XYY karyotype have normal phenotype. This disorder seems associated with a higher risk of developing behavioral and cognitive problems, tall stature, and infertility in adulthood. Sexual development disorder is a rare finding. We report a first case with an abnormal left coronary artery originating from the pulmonary artery in a 47XYY patient.Case. A one-month-old child was referred for ectopic testis and micropenis. Physical examination revealed facial dysmorphia, micropenis, and curvature of the penis with nonpalpable testis. Laboratory tests showed decreased total testosterone and anti-Mullerian hormone (AMH) levels. Blood karyotyping revealed a 47XYY chromosomal formula. At the age of 3 months, the patient developed dyspnea and tachycardia. Echocardiography revealed an anomalous left coronary artery from pulmonary artery with left ventricular dysfunction requiring surgical revascularization by direct reimplantation of the left coronary artery system. Our second case was a 3-year-old child referred for hypospadias with nonpalpable left testicle. Physical examination showed hypertelorism. Blood karyotyping revealed a 47XYY chromosomal formula.Conclusion. To our knowledge, this is the first case of 47XYY syndrome associated with this congenital heart malformation and a sexual development disorder.

Repair of Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA) beyond Infancy

2013 ◽  
Vol 16 (4) ◽  
pp. 210 ◽  
Author(s):  
Sachin Talwar ◽  
Aandrei Jivendra Jha ◽  
Shiv Kumar Choudhary ◽  
Saurabh Kumar Gupta ◽  
Balram Airan
Keyword(s):  
Coronary Artery ◽  
Pulmonary Artery ◽  
Respiratory Tract ◽  
Left Coronary Artery ◽  
Nyha Class ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Anomalous Left Coronary Artery ◽  
Tract Infections ◽  
Anomalous Left Coronary

Between January 2002 and December 2012, five patients (4 female) underwent corrective surgery for anomalous left coronary artery from pulmonary artery (ALCAPA). They were older than 1 year (range, 3-56 years). One of the 2 patients younger than 10 years had presented with congestive heart failure, and the other had experienced repeated episodes of lower respiratory tract infection since childhood. Of the remaining 3 adult patients, 2 had experienced angina with effort, and 1 patient had had repeated respiratory tract infections since childhood, with mild dyspnea on effort of New York Heart Association (NYHA) class II. Three patients had the anomalous left coronary artery implanted directly into the ascending aorta via coronary-button transfer, and 2 patients underwent coronary artery bypass with obliteration of the left main ostium. Two patients underwent concomitant mitral valve repair procedures, and 1 patient underwent direct closure of a perimembranous ventricular septal defect. Four patients survived the surgery, and 1 patient died because of a persistently low cardiac output. Follow-up times ranged from 3 months to 4 years. All survivors are in NYHA class I and have left ventricular ejection fractions of 45% to 60%, with moderate (n = 1), mild (n = 1), or no (n = 2) mitral insufficiency. We conclude that a few naturally selected patients with ALCAPA do survive beyond infancy and can undergo establishment of 2 coronary systems with satisfactory results.

Outcomes of surgical repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children

2021 ◽  
pp. 1-6
Author(s):  
Tong Feng ◽  
Guo Zhangke ◽  
Bai Song ◽  
Fan Fan ◽  
Zhen Jia ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Mitral Regurgitation ◽  
Ejection Fraction ◽  
Pulmonary Artery ◽  
Left Coronary Artery ◽  
Surgical Repair ◽  
Left Ventricular ◽  
Anomalous Origin ◽  
Dimension Index

Abstract Objectives: Anomalous origin of the left coronary artery from the pulmonary artery is associated with high mortality if not timely surgery. We reviewed our experience with anomalous origin of the left coronary artery from the pulmonary artery to assess the preoperative variables predictive of outcome and post-operative recovery of left ventricular function. Methods: A retrospective review was conducted and collected data from patients who underwent anomalous origin of the left coronary artery from the pulmonary artery repair at our institute from April 2005 to December 2019. Left ventricular function was assessed by ejection fraction and the left ventricular end-diastolic dimension index. The outcomes of reimplantation repair were analysed. Results: A total of 30 consecutive patients underwent anomalous origin of the left coronary artery from the pulmonary artery repair, with a median age of 14.7 months (range, 1.5–59.6 months), including 14 females (46.67%). Surgery was performed with direct coronary reimplantation in 12 patients (40%) and the coronary lengthening technique in 18 (60%). Twelve patients had concomitant mitral annuloplasty. There were two in-hospital deaths (6.67%), no patients required mechanical support, and no late deaths occurred. Follow-up echocardiograms demonstrated significant improvement between the post-operative time point and the last follow-up in ejection fraction (49.43%±19.92% vs 60.21%±8.27%, p < 0.01) and in moderate or more severe mitral regurgitation (19/30 vs 5/28, p < 0.01). The left ventricular end-diastolic dimension index decreased from 101.91 ± 23.07 to 65.06 ± 12.82 (p < 0.01). Conclusions: Surgical repair of anomalous origin of the left coronary artery from the pulmonary artery has good mid-term results with low mortality and reintervention rates. The coronary lengthening technique has good operability and leads to excellent cardiac recovery. The decision to concomitantly correct mitral regurgitation should be flexible and be based on the pathological changes of the mitral valve and the degree of mitral regurgitation.

scholarly journals Anomalous Origin of the Left Anterior Descending Coronary Artery in an Adult

2021 ◽  
Vol 10 (01) ◽  
pp. e9-e10
Author(s):  
Keisuke Shibagaki ◽  
Chikara Shiiku ◽  
Hiroyuki Kamiya ◽  
Yoichi Kikuchi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Left Coronary Artery ◽  
Internal Thoracic Artery ◽  
Artery Bypass ◽  
Anomalous Origin ◽  
Bypass Grafting

AbstractAn anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease. Among the variants, an anomalous origin of the left anterior descending coronary artery from the pulmonary artery (ALADPA) is extremely rare. Here, we report a case of ALADPA in an adult that was treated with coronary artery bypass grafting using the left internal thoracic artery.

scholarly journals Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery As a Rare Cause of Left Ventricular Dysfunction

2019 ◽  
Vol 12 (12) ◽  
Author(s):  
Ligia Lopes Balsalobre Trevizan ◽  
Amit Nussbacher ◽  
Maria Carolina Bueno da Silva ◽  
Walther Yoshiharu Ishikawa ◽  
Sergio Almeida de Oliveira ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Pulmonary Artery ◽  
Left Ventricular Dysfunction ◽  
Left Coronary Artery ◽  
Ventricular Dysfunction ◽  
Left Ventricular ◽  
Anomalous Origin

Role of speckle tracking echocardiography in the assessment of post-repair left ventricular function in patients with late presentation of anomalous origin of the left coronary artery from the pulmonary artery

2014 ◽  
Vol 25 (5) ◽  
pp. 969-975 ◽  
Author(s):  
Gholamhosein Ajami ◽  
Mohammad R. Edraki ◽  
Ali R. Moarref ◽  
Ahmad A. Amirghofran ◽  
Mohammad Borzouee ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Left Ventricle ◽  
Pulmonary Artery ◽  
Speckle Tracking ◽  
Left Coronary Artery ◽  
Speckle Tracking Echocardiography ◽  
Left Ventricular ◽  
Doppler Imaging ◽  
Anomalous Origin ◽  
Control Group

AbstractThe aim of this study was to determine the left ventricular myocardial deformation and segmental myocardial dysfunction by speckle tracking echocardiography and tissue Doppler imaging among the operated patients with anomalous origin of the left coronary artery from the pulmonary artery. The study was conducted on 12 patients diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, who had been operated upon between 2001 and 2013 at the medical centres of Shiraz University of Medical Sciences, Shiraz, Iran. The mean age of the patients at the time of surgical correction was 12.6 years ranging from 6 months to 43 years, and the duration of postoperative follow-up was between 1 and 12 years. Comparison of the strain rate between the patients with acceptable ejection fraction and the control group by tissue Doppler imaging showed significant differences between the two groups regarding the lateral wall (p<0.001), but not the septal wall of the left ventricle (p=0.65). Moreover, the strain values by the speckle tracking method revealed significant differences between the patient and the control group regarding the global strain (p=0.016) and anterior, lateral, and posterior segments of the left ventricle. Although postoperative conventional echocardiography revealed normal global left ventricular function with acceptable ejection fraction, abnormal myocardial deformation of the variable segments of the left ventricle with regional and global myocardial dysfunction were well defined by speckle tracking echocardiography.

Sarcoma of the mitral valve causing coronary arterial occlusion in children

2001 ◽  
Vol 11 (5) ◽  
pp. 539-542 ◽  
Author(s):  
Doff B. McElhinney ◽  
David F. Carpentieri ◽  
Nancy D. Bridges ◽  
Bernard J. Clark ◽  
J. William Gaynor ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Mitral Valve ◽  
Left Coronary Artery ◽  
Myogenic Differentiation ◽  
Arterial Occlusion ◽  
Left Ventricular ◽  
Orthotopic Heart Transplantation ◽  
Cardiac Tumors ◽  
Primary Tumors ◽  
First Case

Primary tumors of the cardiac valves are rare. One of the most common reasons that left-sided cardiac tumors come to clinical attention is embolization to the systemic circulation. We present two children who suffered left coronary arterial occlusion due to embolization of a sarcoma of the mitral valve. A 6-year-old female who had been admitted to the hospital after cerebrovascular embolization of a fragment of sarcoma of the mitral valve experienced sudden cardiovascular collapse due to occlusion of the left coronary artery. She was placed on extracorporeal membrane oxygenation, and underwent coronary embolectomy and resection of the tumor from the mitral valve and its tendinous cords. Left ventricular function did not improve, and she underwent orthotopic heart transplantation. On follow-up 32 months after transplant, the patient is well, with no evidence of recurrence of or metastasis from the tumor. The tumor arose from the leaflets and tendinous cords of the mitral valve, and was composed grossly of multiple white nodules. Histopathologic evaluation disclosed fragments composed predominantly of peripheral spindle cells in an extensive fibromyxoid stroma. The mildly pleomorphic cells of the tumor gradually blended with adjacent pieces of the mitral valvar leaflet and tendinous cords. Immunohistochemical studies revealed strong staining for vimentin, smooth muscle actin, muscle specific actin, and myoglobin, suggesting myogenic differentiation. The other patient was a 2½-year-old female who died suddenly at home. Grossly and histologically, the tumor was essentially identical to the first case, and there was a 3 cm string-like extension passing into the orifice of the left coronary artery. To put the cases in context, we compare them with other descriptions of this rare type of tumor.

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