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.

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.

scholarly journals Thrombotic occlusion of the main stem of the left coronary artery in a neonate

1999 ◽  
Vol 9 (2) ◽  
pp. 189-191 ◽  
Author(s):  
Luby Abdurrahman ◽  
Steven M. Schwartz ◽  
Robert H. Beekman
Keyword(s):  
Myocardial Infarction ◽  
Coronary Artery ◽  
Cardiogenic Shock ◽  
Left Coronary Artery ◽  
Arterial Occlusion ◽  
Systemic Circulation ◽  
Left Ventricular ◽  
Thrombotic Occlusion ◽  
Left Ventricular Output ◽  
Patent Arterial Duct

AbstractThrombotic coronary arterial occlusion, and myocardial infarction, are rare in the newborn. We report such a happening presenting shortly after birth with cardiogenic shock, no left ventricular output and a systemic circulation dependent on flow from a patent arterial duct.

scholarly journals Do patients with anomalous origin of the left coronary artery benefit from an early repair of the mitral valve?

2019 ◽  
Vol 57 (1) ◽  
pp. 72-77 ◽  
Author(s):  
Viktoria H M Weixler ◽  
David Zurakowski ◽  
Christopher W Baird ◽  
Alvise Guariento ◽  
Breanna Piekarski ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Mitral Valve ◽  
Pulmonary Artery ◽  
Left Coronary Artery ◽  
Left Ventricular ◽  
Anomalous Origin ◽  
Lv Function ◽  
Z Scores ◽  
Group A ◽  
Group B

Abstract OBJECTIVES The aim of this study was to determine mid-term outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery undergoing coronary repair only (group A) or simultaneous mitral valve repair (group B). METHODS Patients with anomalous origin of the left coronary artery from the pulmonary artery who underwent surgery from 2000 to 2017 were reviewed. Mitral regurgitation (MR) grade (none, mild, moderate, severe), left ventricular (LV) function [ejection fraction (EF): <40%, 40–50%, >50%] and LV Z-scores (long axis) were assessed preoperatively and at last visit. Outcomes were compared within/between the groups using the Wilcoxon signed-rank test. RESULTS Of 58 patients (67% women; median age 4.4 months), 39 patients were in group A (67%) and 19 patients in group B (33%). The median hospital stay (11 days, interquartile range 5–18) and average follow-up time (2.6 ± 0.5 years) did not differ significantly between the groups (P > 0.05). Four patients in group A (10.3%) underwent mitral valve reintervention. The median MR grade differed significantly between the groups preoperatively (2 vs 3, P < 0.001) but not at the last visit (2 vs 2, P = 0.88); both groups improved significantly (P = 0.021, P < 0.001). EF grade (<40%, 40–50%, >50%) did not differ significantly between the groups at baseline (group A: 38%/23%/38% vs group B: 58%/10%/32%, P = 0.32) or at last visit (group A: 18%/15%/67% vs group B: 26%/16%/58%, P = 0.75); both groups improved significantly (P = 0.004, P = 0.014). The mean LV Z-scores for groups A and B were 3.1 ± 0.5 and 4.5 ± 0.6 before surgery (P < 0.05) and 1.5 ± 0.3 and 2.7 ± 0.6 at last visit (P = 0.77). CONCLUSIONS The repair of anomalous origin of the left coronary artery from the pulmonary artery is associated with improvement in MR, EF and LV dimensions. However, in cases of ≥moderate MR, the risk of mitral valve reintervention may be higher in patients undergoing coronary transfer only.

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 coronary artery from the pulmonary artery: late results with special attention to the mitral valve☆

2009 ◽  
Vol 36 (2) ◽  
pp. 244-249 ◽  
Author(s):  
Walid Ben Ali ◽  
Olivier Metton ◽  
François Roubertie ◽  
Philippe Pouard ◽  
Daniel Sidi ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Mitral Valve ◽  
Pulmonary Artery ◽  
Left Coronary Artery ◽  
Late Results ◽  
Anomalous Origin

scholarly journals Left anterior descending coronary artery dissection during ventricular tachycardia ablation – case report

2018 ◽  
Vol 56 (1) ◽  
pp. 63-66
Author(s):  
Kresimir Kordic ◽  
Sime Manola ◽  
Ivan Zeljkovic ◽  
Ivica Benko ◽  
Nikola Pavlovic
Keyword(s):  
Ventricular Tachycardia ◽  
Coronary Artery ◽  
Left Main Coronary Artery ◽  
Left Ventricular ◽  
Artery Dissection ◽  
Complication Rates ◽  
Left Main ◽  
Coronary Artery Dissection ◽  
Ventricular Tachycardia Ablation ◽  
First Case

Abstract Fascicular left ventricular tachycardia (VT) is the second most frequent idiopathic left VT in the setting of a structurally normal heart. Catheter ablation is curative in most patients with low complication rates. We report a case of ostial left anterior descending coronary artery (LAD) occlusion during fascicular ventricular tachycardia ablation. Dissection was the most likely cause of LAD obstruction. To the authors’ best knowledge, this is the first case reporting selective LAD dissection during electrophysiology study with no left main coronary artery (LMCA) affection.

scholarly journals Induction of myocardial infarcts of a predictable size and location by branch pattern probability-assisted coronary ligation in C57BL/6 mice

2004 ◽  
Vol 286 (3) ◽  
pp. H1201-H1207 ◽  
Author(s):  
Dongchoon Ahn ◽  
Linda Cheng ◽  
Chanil Moon ◽  
Harold Spurgeon ◽  
Edward G. Lakatta ◽  
...  
Keyword(s):  
Heart Disease ◽  
Coronary Artery ◽  
Left Coronary Artery ◽  
Left Ventricular ◽  
Myocardial Infarctions ◽  
Branch Points ◽  
Left Main ◽  
Uniform Size ◽  
Disease Research ◽  
Coronary Ligation

The ability to create experimental myocardial infarctions of reproducible size and location is tantamount to progress in multiple facets of ischemic heart disease research. Branches of the mouse left main descending coronary artery penetrate the myocardium close to their origin and require “blind” ligation. Our objective was to develop a technique for ligation of nonvisible coronary artery branches to permit the reliable creation of infarcts of uniformly small size and location. From latex castings of the left coronary artery of C57BL/6J mice ( n = 53), we calculated the highest probability for the location of branch points of two of three left ventricular (LV) branches distal to the origin of the left main descending artery. On the basis of these anatomic probabilities, we blindly ligated two areas that were likely to be locations of these nonvisible LV branches. We were successful in producing two types of small transmural myocardial infarctions (16.04 ± 3.64 and 4.68 ± 1.47% of the LV) in 57% of attempts. Thus our branch pattern probability-assisted method permits routine creation of small infarcts of uniform size in the mouse.

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