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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To analyze the clinical significance of eccentric aortic regurgitation (AR) complicating the right coronary cusp prolapse associated with a ventricular septal defect (VSD), the Doppler echocardiograms of 129 patients were reviewed. In 102 patients, AR was classified as mild and in 27 patients it was classified as moderate. Eccentric AR was defined as the jet distributing in an eccentric direction. In 15 patients of the moderate group, AR was already moderate at the initial examination and of these, the AR was eccentric in 14 and central in 1. In 12 patients who initially had mild AR, it became moderate during follow-up. In 7 patients with mild, central AR, 6 worsened to central moderate AR and 1 evolved to eccentric moderate AR. Eccentric mild AR patients all developed eccentric moderate AR. Within the mild AR group, 5 of 9 patients with eccentric AR progressed from mild to moderate, whereas only 7 of 105 patients with central AR did so (p<0.01). In conclusion, eccentric AR may be an advanced finding of the AR associated with right coronary cusp prolapse in some patients, but in others eccentric AR is highly likely to progress and is malignant.
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PMID:Eccentric aortic regurgitation in patients with right coronary cusp prolapse complicating a ventricular septal defect. 1289 Sep 8

We describe the phenotype of a 5 year old girl with features resembling Temtamy syndrome, including agenesis of the corpus callosum, ventriculomegaly, frontal bossing, peaked eyebrows, ptosis, malformed and low set ears, a depressed nasal bridge, a long philtrum, and iris and chorioretinal colobomas. Features unique to this child include profound mental retardation, bilateral sensorineural hearing loss, agenesis of the corpus callosum, patent ductus arteriosus, ventricular septal defect, unilateral renal agenesis, neurogenic bladder and hydronephrosis. High resolution chromosome analysis demonstrated a de novo, balanced translocation [46,XX,t(2;9)(p24;q32)]; and her case has some overlapping phenotypic features with cases of monosomy for 2p. This is the first documented case of Temtamy syndrome with a specific chromosomal anomaly, and will assist with the elucidation of the syndrome's underlying genetic defect.
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PMID:Temtamy-like syndrome associated with translocation of 2p24 and 9q32. 1456 55

Percutaneous closure of perimembranous ventricular septal defects (VSDs) has been feasible, safe, and effective with the new Amplatzer membranous septal occluder. We report further experience with this device with emphasis on morphological aspects of the VSDs and technical issues. Ten patients (median age and weight, 14 years and 34.5 kg, respectively) with volume-overloaded left ventricles underwent closure under general anesthesia and transesophageal guidance (TEE). The VSD diameter was 7.1 +/- 4.0 mm by angiography and 7.8 +/- 3.7 mm by TEE. Three patients had defects associated with aneurysm-like formations (two with multiple exit holes), four had defects shrouded by extensive tricuspid valve tissue, two had defects with little or no tricuspid valve involvement, and one had a right aortic cusp prolapse with trivial aortic regurgitation. Implantation was successful in all patients, although in two the initial device had to be changed for a larger one. Kinkings in the delivery sheath, inability to position the sheath near the left ventricular apex, and device prolapse through the VSD prompted modifications in the standard technique of implantation. Device orientation was excellent except in one case. Nine patients had complete occlusion within 1-3 months. Device-related aortic or tricuspid insufficiency, arrhythmias, and embolization were not observed. Two patients had slight gradients across the left ventricular outflow tract, normalizing after 3 months. The Amplatzer membranous septal occluder was suitable to close a wide range of perimembranous VSD sizes and morphologies with good short-term outcomes. Longer follow-up is required.
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PMID:Percutaneous closure of perimembranous ventricular septal defects with the Amplatzer device: technical and morphological considerations. 1498 5

We report a case of unilateral congenital ptosis which is associated with ocular and systemic congenital malformations including mild microphthalmia, microcornea, cataract, iris and chorioretinal coloboma, ectopic kidney, and ventricular septal defect. An inciting factor, acting during the second month of gestation, may affect the development of the eye, heart, and abdomen and may lead to congenital malformations. Although congenital ptosis rarely presents with ocular and systemic congenital malformations, ophthalmologists should be alert for the possibility of coexisting structural defects. Congenital ptosis is a muscular dystrophy demonstrated by various degrees of muscular degeneration and it may rarely be associated with ocular and systemic congenital malformation. Here, we report a case of congenital ptosis associated with more than one ocular and systemic malformation.
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PMID:Congenital ptosis and associated congenital malformations. 1522 38

We report the case of a Caucasian man with the unusual combination of an infundibular ventricular septal defect (VSD), an aneurysm of the sinus of Valsalva communicating with the right ventricle, and a bicuspid aortic valve. When aortic valve regurgitation associated with mild right aortic cusp prolapse appeared during follow-up examination, the patient, although totally asymptomatic, underwent surgical repair of the aortic sinus and closure of the VSD.
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PMID:Infundibular ventricular septal defect, aneurysm of the sinus of Valsalva, and bicuspid aortic valve in a caucasian male. 1574 18

An 18 month-old girl was diagnosed as ventricular septal defect (VSD) with mild aortic valve prolapse. She underwent a closure of VSD. Intra-and early postoperative course was uneventful. However, 20 hours after surgery, sudden bradycardia led to cardiac arrest and strong muscle rigidity was seen. Hyperkalemia and metabolic acidosis rapidly progressed and resuscitation was failed. Extracorporeal life support and continuous hemodialysis were initiated, but the patient died with multiple organ failure on 5th postoperative day. Her clinical course supported the diagnosis of delayed onset malignant hyperthermia. Histopathological findings of muscle biopsy were consistent with rhabdomyolysis, and immunopathological stains demonstrated changes as in a Duchenne type muscular dystrophy carrier. Delayed onset malignant hyperthermia is an extremely rare complication of general anesthesia. We should be aware of this lethal condition, which occurs with a certain time lag after surgery, especially when the patient has possible background of myopathy.
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PMID:[Delayed onset malignant hyperthermia after a closure of ventricular septal defect]. 1577 37

Coronary artery fistulas vary widely in their morphological appearance and presentation. These fistulas are congenital or acquired coronary artery abnormalities in which blood is shunted into a cardiac chamber, great vessel, or other structure, bypassing the myocardial capillary network. The majority of these fistulas arise from the right coronary artery and the left anterior descending coronary artery; the circumflex coronary artery is rarely involved. Clinical manifestations vary considerably and the long-term outcome is not fully known. The patients with coronary fistulas may present with dyspnea, congestive heart failure, angina, endocarditis, arrhythmias, or myocardial infarction. A continuous murmur is often present and is highly suggestive of a coronary artery fistula. Differential diagnosis includes persistent ductus arteriosus, pulmonary arteriovenous fistula, ruptured sinus of Valsalva aneurysm, aortopulmonary window, prolapse of the right aortic cusp with a supracristal ventricular septal defect, internal mammary artery to pulmonary artery fistula, and systemic arteriovenous fistula. Although noninvasive imaging may facilitate the diagnosis and identification of the origin and insertion of coronary artery fistulas, cardiac catheterization and coronary angiography is necessary for the precise delineation of coronary anatomy, for assessment of hemodynamics, and to show the presence of concomitant atherosclerosis and other structural anomalies. Treatment is advocated for symptomatic patients and for those asymptomatic patients who are at risk for future complications. Possible therapeutic options include surgical correction and transcatheter embolization. Historical perspectives, demographics, clinical presentations, diagnostic evaluation, and management of coronary artery fistula are elaborated.
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PMID:Coronary artery fistulas: clinical and therapeutic considerations. 1612 61

This study reports on experience with transcatheter closure of congenital ventricular septal defects (VSDs) with Amplatzer septal occluders. From January 2000 to April 2005, transcatheter Amplatzer device implantation was attempted in 122 patients with congenital VSD (30 with muscular, 87 with perimembranous, and 5 with residual postsurgical repair of conotruncal malformations). Patient mean age was 15 years (range, 6 months to 64 years), and mean weight was 35 kg (range, 5.8 to 102 kg). The VSD mean size was 7 mm (range, 4 to 16 mm), mean Qp/Qs was 2.1 (range, 1.3 to 4), and mean fluoroscopy time was 32 minutes (range, 5 to 129 minutes). All procedures were performed with the patient under general anesthesia and guided by fluoroscopy and transesophageal echocardiography. The device size chosen was usually 1- to 2-mm larger than the maximum defect size as assessed by either the echocardiographic or angiographic views that were judged most reliable. Amplatzer muscular devices were placed in 47 patients, and the membranous devices were placed in 72 patients. The procedure was not performed in 3 patients with perimembranous VSD because of the impossibility of achieving an adequate long sheath position in 1 patient, onset of complete atrioventricular (AV) block during catheter manipulation in 1 patient, and the presence of aortic valve prolapse preventing a safe device placement in 1 patient. Satisfactory device implantation was achieved in 119 of 122 patients (97.5%): a tiny smoke-like residual flow through the device was often seen immediately after the procedure (50%); residual shunting was detectable in 19% after 24 hours and in only 4% at 6 months. The following additional catheter interventions were performed simultaneously: balloon pulmonary valvuloplasty in 3 patients, device closure of atrial septal defects in 2 patients, coil occlusion of the arterial duct in 1 patient, stenting coarctation in 1 patient, and stenting of the right pulmonary artery in 1 patient. Minimal aortic regurgitation developed in 3 patients, and minimal tricuspid regurgitation in 3 patients; no patient required additional treatment. Device embolization occurred in 3 patients (1 patient with muscular VSD, 2 with perimembranous VSD); catheter retrieval and implantation of a second device was successfully performed in all patients. Transient left bundle branch block occurred in 2 patients, and transient first-degree AV block in 1 patient. Among the perimembranous VSD cases, complete AV block occurred acutely (within 48 hours) in 3 patients, requiring a pacemaker in 1 patient; complete heart block occurred in the other 2 patients after 5 and 12 months, requiring pacemakers. There was no mortality. Transcatheter closure of muscular and perimembranous VSDs offers encouraging results: 96% complete closure at midterm follow-up. Complications are limited; the most relevant appears to be device-related complete heart block in perimembranous VSD. Greater experience and long-term follow-up are required to assess the safety and effectiveness of this procedure as an alternative to conventional surgery.
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PMID:Transcatheter closure of congenital ventricular septal defect with Amplatzer septal occluders. 1639 93

Rupture of a sinus of Valsalva aneurysm (SVA) is a rare, but life-threatening, event and requires immediate recognition and intervention. We present two previously healthy and physically active patients who were 12 and 33 years of age when rupture of a right coronary SVA into the right ventricle occurred. A subarterial ventricular septal defect (VSD) was detectable in both patients. Cardiac surgery involved VSD closure as well as reconstruction of the aortic valve. Considering complications of subarterial VSD, such as aortic cusp prolapse, aortic insufficiency or SVA, we suggest close follow-up and surgical closure of the VSD in case of any aortic valve deformity.
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PMID:Ruptured sinus of Valsalva aneurysm in two patients with subarterial ventricular septal defect. 1659 24

An 8-month-old entire Miniature Dachshund, weighing 4.2 kg, was presented for examination following delvelopment of a cough. Ventricular septal defect had been diagnosed tentatively in its infancy on the basis of a cardiac murmur detected by auscultation and echocardiography. Echocardiography using a B mode right parasternal long-axis view showed a defect at the atrioventricular junction and a thickened cusp of the aortic valve prolapsing into the defect. Colour-flow Doppler showed shunt blood flow across the defect at the level of the atrioventricular junction, from left to right. The sinus of Valsalva was dilated, with turbulent blood flow. Aortic regurgitation was also observed. Cardiac catheterisation studies confirmed the diagnosis of a supracristal ventricular septal defect with aortic regurgitation. Despite medication with digoxin, enalapril and aminophylin, started from the first admission, left ventricular internal dimensions gradually increased, and fractional shortening of the left ventricle gradually decreased. Surgery, with the aid of extracorporeal circulation, to close the ventricular septal defect, was performed 1 year after the initial examination. The aortic valve was left untreated. Postoperatively, the systolic murmur disappeared. Shunt flow from the left to the right ventricle was no longer observed on echocardiography, however there was still a small amount of aortic regurgitation during diastole visualised with colour-flow Doppler echocardiography. The prolapse of the cusp of the aortic valve on B-mode echocardiography was no longer observed and thickening of the cusp had not progressed. Left ventricular function measurement using M mode echocardiography showed a reduced left ventricular volume overload with reduced left ventricular internal dimensions and increased fractional shortening. The cough was relieved and no follow-up medication was scheduled. Early surgical closure of the ventricular septal defect improved the patient's condition and controlled prolapse and thickening of the aortic valve.
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PMID:Surgical correction of ventricular septal defect with aortic regurgitation in a dog. 1662 90


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