1/51. Heart/single-lung transplant for a "failed Fontan" with pulmonary A-V malformation.Pulmonary arteriovenous malformation can occur in patients with functional single ventricle after a cavopulmonary connection. There is no effective medical treatment for this complication. Pulmonary arteriovenous malformations may regress over time after heart transplantation, but may be a source of significant perioperative morbidity. We report the case of a woman with single ventricle, ventricular dysfunction, and bilateral pulmonary arteriovenous malformations who had successful treatment of both cardiac and pulmonary failure with en bloc heart and right lung transplantation.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
2/51. Noncompaction of the ventricular myocardium.Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular recesses communicating with the left ventricular cavity. The disease uniformly affects the left ventricle, with or without concomitant right ventricular involvement, and results in systolic and diastolic ventricular dysfunction and clinical heart failure. Noncompaction was initially described in children. However, recent studies have characterized this disease in the adult population, in whom this process may be more prevalent than currently appreciated. We describe an illustrative case of isolated noncompaction of the ventricular myocardium in a 57-year-old woman with the typical clinical and echocardiographic features of the disease. The literature on the topic is reviewed.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
3/51. Isolated noncompaction of the myocardium: an exceedingly rare cardiomyopathy. A case report.Isolated noncompaction of the left ventricular myocardium is a rare cardiac disorder due to an arrest in myocardial morphogenesis. It is characterized by prominent and excessive trabeculation in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Echocardiographic findings are important clues for the diagnosis. Clinical symptoms include signs of left ventricular systolic dysfunction even to the point of heart failure, ventricular arrhythmias, and embolic events. We describe an adult case in whom the only clinical symptoms were life-threatening ventricular arrhythmias. Transthoracic echocardiography did not contribute to the diagnosis, which was made thanks to left ventricular contrast angiography. Electrophysiological testing induced a fast monomorphic sustained ventricular tachycardia, with hemodynamic impairment, that was refractory to pharmacological treatment, and for this reason a permanent cardioverter-defibrillator was implanted. A subsequently performed transesophageal echocardiographic examination showed a localized, regional increase in left ventricular wall thickness and degree of trabeculation. The causes and electrophysiological mechanisms of arrhythmias in noncompaction are still unknown: grossly irregular branching and connecting of myocardial fascicles in the noncompacted segments, isometric contraction with increased wall stress, and localized coronary perfusion impairment can all induce disorganized or delayed activation and increase the potential for arrhythmias. This is the first reported case of noncompaction in which an implantable defibrillator was used to control life-threatening arrhythmias.- - - - - - - - - - ranking = 0.0056207186681318keywords = dysfunction (Clic here for more details about this article) |
4/51. Neurologic and gastrointestinal dysfunction in cardio-facio-cutaneous syndrome: identification of a severe phenotype.Controversy exists over the distinction between cardio-facio-cutaneous (CFC) syndrome and noonan syndrome (NS). Several authors have suggested that they are different phenotypes of the same condition. We present the cases of two patients with CFC syndrome to show that it is a distinct condition with a unique combination of findings and a more complex natural history. These patients, both girls, were born with signs of fetal edema following pregnancies complicated by polyhydramnios. Each has short stature with relative macrocephaly; fuzzy, sparse hair; and the typical craniofacial features, including a square forehead. Both have heart abnormalities, failure to thrive, and severe feeding problems requiring gastrostomy. They are markedly hypotonic and developmentally delayed. They show signs of frequent eyelid fluttering and have oral aversion, tactile hypersensitivity, and sensory integration abnormalities. keratosis pilaris, the characteristic skin symptom, is also present in both patients. In a review we identified 56 cases of CFC syndrome. We scored these cases by 10 clinical criteria and identified a subset with a specific, severe phenotype distinct from that of NS. The serious neurologic and gastrointestinal complications, in addition to the skin abnormalities and characteristic facies in this group, clearly separate these patients from the mildly affected ones, most of whom appear to have NS or another syndrome. We discuss the differences between the severe CFC phenotype and those of overlapping conditions. We set forth stringent diagnostic criteria for CFC syndrome, the initial step toward identifying a molecular basis for this condition.- - - - - - - - - - ranking = 0.022482874672527keywords = dysfunction (Clic here for more details about this article) |
5/51. arthrogryposis, renal dysfunction and cholestasis syndrome.We report for the first time from the Arabian Gulf area 3 patients with arthrogryposis multiplex congenita, cholestasis and renal tubular dysfunction from a Saudi family with 2 other siblings and 3 cousins who possibly died with a similar clinical picture. We also document for the second time in literature other findings in this syndrome including cerebral abnormalities (hypoplastic corpus callosum), congenital heart disease and nerve deafness. We suggest that some of these cases might benefit from ursodeoxycholic acid therapy. We believe that this autosomal recessive disorder is possibly under-diagnosed in this region with a high consanguineous marriage rate.- - - - - - - - - - ranking = 0.028103593340659keywords = dysfunction (Clic here for more details about this article) |
6/51. Corrected transposition of the great arteries with several associated anomalies in a 68-year-old patient.Few patients with corrected transposition of the great arteries survive past 50 years of age because of the association with congenital defects, development of total atrioventricular block, and right ventricular dysfunction. We report the case of a male patient with dextrocardia in situs solitus and corrected transposition of the great arteries associated with a wide atrial septal defect and severe pulmonary valvar and subvalvar stenoses. The patient also developed a large aneurysm on the pulmonary artery, total atrioventricular block diagnosed 8 years earlier, symptoms of dysfunction of the systemic ventricle in the previous 2 years, insufficiency of the left atrioventricular valve, and aortic regurgitation. Despite all these associated anomalies, the patient developed class III cardiac decompensation only at the age of 68 years, which makes this case a rarity. The patient was clinically treated, and was discharged from the hospital in good condition.- - - - - - - - - - ranking = 1.0056207186681keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
7/51. Pathogenesis of acardiac twinning: clues from an almost acardiac twin.OBJECTIVE: To search for clues to the pathogenesis of acardiac twinning. methods: We examined a case of monoamniotic twins in which twin A's only sonographic abnormality was a dilated, tortuous ductus venosus. Twin B also had this abnormality as well as multiple other anomalies that included enormous hydrops and a severely hypoplastic heart. Following termination of pregnancy, autopsy was performed. RESULTS: Postmortem examination of the placenta confirmed monochorionic, monoamniotic placentation with two adjacent trivascular cords. autopsy confirmed the sonographic findings of enormous hydrops in twin B with a severely malformed, almost nonexistent heart. In addition, the liver was small and was represented by a cyst-like structure with thin rims of congested parenchyma surrounding large vascular spaces. CONCLUSION: We believe the sequence of events in this case was early twin-to-twin transfusion resulting in a dysfunctional heart in twin B. This enabled a twin reversal arterial perfusion sequence with further deterioration of twin B's heart and extreme congestion of deoxygenated blood exiting the heart into the inferior vena cava and ductus venosus. This case supports the concept that circulatory reversal in the face of an initially functioning heart may lead to congestion, tissue hypoxia and secondary organ atrophy.- - - - - - - - - - ranking = 0.0056207186681318keywords = dysfunction (Clic here for more details about this article) |
8/51. Isolated non-compaction of the myocardium diagnosed in the fetus: two sporadic and two familial cases.Isolated non-compaction of the ventricular myocardium is characterized by numerous and prominent trabeculations and deep intertrabecular recesses. This rare disease is due to an arrest of myocardial morphogenesis. Most cases, when seen in children, are associated with obstructive malformations. Isolated non-compaction is even rarer in childhood, and affects predominantly the myocardium of the left ventricle. morbidity and mortality resulting from cardiovascular complications is high. In most cases, transplantation is the final option. To our knowledge, this rare cardiac malformation has yet to be diagnosed in the fetus. We report here two sporadic cases, one male and one female, and 2 familial cases, both male, which were diagnosed prenatally and followed by fetal echocardiography. Our study indicates that isolated non-compaction is a primary disorder of early fetal development. Our cross-sectional echocardiographic examinations revealed a fetal cardiomyopathy, with prominent and numerous trabeculations and deep intertrabecular recesses of the myocardium at the apex of the ventricles. In contrast with postnatal experience, we found isolated non-compaction mostly in the right ventricle. Systolic dysfunction was found in all cases. The diagnosis was confirmed by histology in 3 fetuses dying with cardiac failure, and by postnatal cross-sectional echocardiography in the fetus who survived. Two male fetuses belonged to a family in which 3 individuals were subsequently found to be affected. We discuss the issues of prenatal diagnosis, natural history, and myocardial histology.- - - - - - - - - - ranking = 0.0056207186681318keywords = dysfunction (Clic here for more details about this article) |
9/51. Coil embolization of a patent ductus venosus in a 52-day-old girl with congenital heart disease.We present the case of 52-day-old girl with a common atrioventricular canal. Severe liver dysfunction persisted following complete repair of the cardiac defect. A patent ductus venosus appeared to be the source of the hemodynamic disturbance responsible for hepatic dysfunction. Given her critical condition, coil embolization of the ductus venosus was performed, after which the patient improved rapidly. The ductus venosus should be tested for patency when liver dysfunction persists after the corrective cardiac surgery, and coil embolization is the treatment of choice in gravely ill children.- - - - - - - - - - ranking = 0.016862156004395keywords = dysfunction (Clic here for more details about this article) |
10/51. Fatal double aortic arch anomaly and maternal cocaine abuse.We report a case of 2 months old female (born to a cocaine abuser mother) having respiratory dysfunction attributed to pneumonia. autopsy, following a sudden, unexpected death, revealed a double aortic arch which was compressing the trachea and the esophagus. health care professionals should conduct a detailed cardiac assessment on all cocaine-exposed infants at the time of birth and during infancy. Double aortic arch should be considered as a possible complication of intrauterine cocaine exposure and may present as sudden death.- - - - - - - - - - ranking = 0.0056207186681318keywords = dysfunction (Clic here for more details about this article) |
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