Repair of double-outlet right ventricle

The results of repair of double outlet right ventricle in 26 children are reported. Eighteen of these patients had subaortic ventricular septal defect (VSD), without pulmonary stenosis (PS) in 5 and with PS in 13. Another 8 patients had subpulmonic VSD, without PS in 6 and with PS in 2. All had concordant atrioventricular relationships. There were six early deaths and two late deaths of the 26 patients in our study. Of these, 5 patients had severe associated cardiac abnormalities, and 2 who died early had had an incision in the systemic (right) ventricle.     

Anatomic features and surgical strategies in double-outlet right ventricle

BACKGROUND: The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality. METHODS AND RESULTS: Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years. CONCLUSIONS: Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.     

Echocardiographic diagnosis of single coronary artery in double-outlet right ventricle

A 90-year-old woman was admitted to our hospital in December 1993 because of dyspnea on exertion and malaise. She had been well until October 1993, when she first noticed Raynaud's phenomenon, skin tightening, digital ulceration and scarring of her hands. On physical examination, generalized edema was found, along with acrosclerosis with contracture, especially in the fingers, wrists, and elbows. Inspiratory crackles were noted. A roentgenogram of the chest and an echocardiogram revealed pulmonary fibrosis, pulmonary congestion, and massive pleural and pericardial effusions. The pleural effusion was a transudate. Progressive systemic sclerosis was diagnosed, and furosemide and isosorbide were given. The edema and pulmonary congestion resolved, but the pleural and pericardial effusions did not. Prednisolone was given, which reduced the pleural effusion but not the pericardial effusin. The pleura and the pericardium are not usually involved in progressive systemic sclerosis, and this disease rarely occurs in patients over 70 years old. To the best of our knowledge, this was one of the oldest patients with progressive systemic sclerosis. The combination of massive pleural and pericardial effusions, and the advanced age of onset make the present case unusual.     

An unusual case of large right atrial myxoma

A case of right atrial myxoma is described. The patient had vague and variable symptoms and it was not until five years after first presentation that she had developed clinical and electrocardiographic evidence of right atrial hypertrophy, and angiocardiographic evidence of a fist-sized myxoma which was subsequently removed surgically.     

Angiographic demonstration of blood supply of right atrial myxoma

A 53-year-old woman underwent cardiac catheterization for assessment of coronary arterial disease. An unexpected finding of a gradient between right atrium and right ventricle led to the discovery of an unsuspected right atrial myxoma. The diagnosis was established by the presence of a filling defect in the right atrial angiocardiogram and by the demonstration of "tumour vessels" by selective right coronary angiography. Selective coronary angiography can visualize the blood supply to an intracardiac tumour and thus confirm the diagnosis preoperatively.     

Simulation of right atrial cardiac myxoma by silent hepatocellular carcinoma

A clinically silent hepatocellular carcinoma presenting as a mixoma of the right atrium is described. Intra-atrial growth has been reported in advanced, clinically manifested cases of liver carcinomas in African and Japanese subjects, but very occasionally in Caucasian people. Our case further suggests that this occurrence should also be considered in Western Countries.     

Acute bicaval obstruction as a result of intracapsular haemorrhage in a right atrial myxoma: report of a case

Right atrial myxomas are rare and presentation is characterised by the gradual onset of one or more of a triad of constitutional, obstructive, or embolic symptoms. We describe a case in which interstitial haemorrhage within a right atrial myxoma resulted in rapid expansion and presentation with features of rapidly progressing bicaval obstruction and atrial flutter. Transthoracic echocardiography failed to detect this tumour; however, transoesophageal echo clearly displayed it and gave information on its attachment and relations which proved to be valuable in the planning of its surgical excision.     

Surgical removal of right atrial myxoma with severe hypoxemia caused by right to left shunt through persistent foramen ovale]

A 41-year-old woman was referred to our hospital due to dyspnea and cyanosis. Echocardiography revealed a right atrial tumor with right to left shunt through PFO. There was no evidence of pulmonary embolism from the tumor. The tumor was removed under cardiopulmonary bypass and PFO was closed. The patient is doing well now.     

Large right ventricular myxoma in a 79-year-old male

A 79-year-old male, admitted because of severe dyspnea on exertion, showed echocardiographic findings of a large tumor in the dilated right ventricle. The right ventricular outflow tract was nearly occluded by the tumor mass, and the mass was attached to the interventricular septum by a pedicle. The tumor removal operation was successful. The size of the tumor was 40 mm x 90 mm, and the weight 70 g. Microscopic findings showed typical myxomatous tissue with high cellularity, and no malignancy was observed. This is the oldest reported patient with right ventricular myxoma which was cured by operation.     

Coronary artery fistula with left atrial myxoma: report of a case

A 64-year-old male was referred for surgical treatment of left atrial myxoma. Preoperative coronary angiography revealed coronary artery fistula from the left anterior descending artery and the circumflex artery draining into the main pulmonary artery. Operative treatment was performed including resection of the myxoma, patch closure of the atrial septal defect, and closure of the fistula with pledgeted mattress sutures from within the main pulmonary artery on cardiopulmonary bypass. His postoperative course was uneventful, and disappearance of the left atrial myxoma and the coronary artery fistula was ascertained by echocardiography and coronary angiography.     

The recurrence of an atrial myxoma in a patient with a mechanical mitral prosthesis--apropos a case

Recurrence of cardiac myxoma after surgery is an uncommon situation, particularly if a wide excision of the tissue under the tumour has been done. The authors report a case of a 54-year-old male presenting with a left atrial myxoma near the mitral valve, which had to be replaced by a mechanical prosthetic valve during the removal of the tumour. One year later, he was admitted to hospital with persistent fever, weight loss, and congestive heart failure. After a positive hemoculture, intravenous antibiotherapy was initiated, and twice modified because of relapsing fever. Six weeks later, he was transferred to our institution, after an episode of severe acute pulmonary edema. 2D-Doppler echocardiography suggested the possibility of prosthesis dysfunction, revealing a transprothetic diastolic flow with a high peak velocity and moderately elevated pressure half-time. No intra-atrial masses were visualized. Computed tomography was also inconclusive, because of multiple artifacts produced by the prosthesis. These results led to the performance of a cardiac catheterization with contrast ventriculography, which revealed the presence of a transprothetic gradient, and mild mitral regurgitation. The patient was submitted to cardiac surgery, which revealed a recurrent pedunculated left atrial myxoma, with mechanical obstruction of the mitral prosthetic valve. No signs of endocarditis were found. Recurrent cardiac myxomas are reviewed and discussed, as well as the specific problems of the present case, namely the presence of a mechanical prosthetic mitral valve and the initial hemoculture results, with consequent diagnostic delay.     

Video-assisted minimal access in excision of left atrial myxoma

BACKGROUND: Minimal access surgery with video-assisted endoscopy has been applied to the correction of intracardiac lesions. We report our experience using this technique in surgical excision of left atrial myxoma in 3 patients. METHODS: From November 1995 to March 1997, 3 female patients, ages 45 to 80 years (mean, 62.7 years), received emergency operations for excision of left atrial myxoma. These operations were performed through a right anterior submammary minithoracotomy or right parasternal incision with the assistance of endoscopy during femoro-femoral cardiopulmonary bypass. The myocardium was protected by continuous coronary perfusion with fibrillatory arrest or cardioplegic arrest with aortic cross-clamping. RESULTS: All the tumors were excised completely through the right atrial approach. The bypass time was 92 to 148 minutes (mean, 111 minutes). The operation time was 3.2 to 4.4 hours (mean, 3.7 hours). There were no hospital deaths. Follow-up, which ranged from 6 to 19 months (mean, 10.5 months), was complete in all patients. Transthoracic echocardiographic examination showed good ventricular function without any residual tumors. Patients were found to be in New York Heart Association functional class I or II. They were satisfied with the good cosmetic healing of the incision. CONCLUSIONS: Our experience demonstrates that minimal access surgery is a technically feasible, safe, and effective procedure in surgical excision of left atrial myxoma.     

Autism and visual agnosia in a child with right occipital lobectomy

Situational factors are important determinants of alcohol and drug use in general samples of substance abusers, with alcohol users and drug users reporting different trigger situations. This study represents an initial attempt to investigate high-risk alcohol and drug use situations in a sample of dually diagnosed individuals. Thirty men and women with both a serious mental illness and an alcohol use or drug use disorder were recruited from an inpatient psychiatric unit and completed questionnaires assessing high-risk alcohol and drug use situations psychiatric symptomatology, and psychological symptoms that trigger substance use. Although the alcohol and drug groups did not differ significantly in the frequency of self-reported high-risk situations for substance use, effect size analyses suggest that six of eight comparisons would demonstrate statistically significant differences with a larger sample size. Both groups reported using substances most frequently when feeling anxiety or depressive symptoms rather than psychotic symptoms. Although preliminary, these results have relevance for the treatment of dually diagnosed persons, particularly for behavioral treatment interventions such as coping skills training and stimulus control techniques.     

Unusual course of right atrial myxoma, masked by acute abdominal pain, and complicated by pulmonary embolus

A 41-year-old female underwent complete resection of a left petroclival meningioma via an anterior transpetrosal approach, during which the greater superficial petrosal nerve was divided. On the 14th day after the operation, she first noticed leakage of clear fluid from her right nostril whenever the ambient room temperature rose. This pseudo-cerebrospinal fluid rhinorrhea may have developed because of parasympathetic hypersensitivity due to division of the greater superficial petrosal nerve.     

Lameness and leg weakness in rapidly growing turkeys associated with hip lesions

A form of epidemic lameness affecting rapidly growing broadbreasted turkeys of different breeds was investigated in 16 flocks. The condition commenced when birds were between eight and 14 weeks old and was consistenly associated with the development of lesions in the hip. Affected birds first showed leg weakness and ataxia and later chronic postural changes involving the hip. The post mortem lesions were those of inflammation, oedema, ulceration, fibrosis and complensatory hypertrophy of localised areas associated with the hip articulation. The cause was not established but the anatomical features of affected birds, their nutritional status and their apparent freedom from recognised infectious diseased are discussed.     

Prevention of recurrent atrial fibrillation with chronic dual-site right atrial pacing

OBJECTIVES: We investigated 1) the feasibility, safety and efficacy of multisite right atrial pacing for prevention of atrial fibrillation (AF); and 2) the ability of atrial pacing in single- and dual-site modes to increase arrhythmia-free intervals in patients with drug-refractory AF. BACKGROUND: We recently developed and applied a novel technique of dual-site right atrial pacing in an unselected group of consecutive patients with AF requiring demand pacing. A prospective crossover study design was used to evaluate single- and dual-site right atrial pacing modes. METHODS: The frequency of AF during the 3 months before pacemaker implantation was analyzed. Consecutive consenting patients underwent insertion of two atrial leads and one ventricular lead with a DDDR pulse generator. Patients were placed in a dual-site pacing mode for the first 3 months and subsequently mode switched to single site pacing for 3 months. Mode switching was repeated at 6-month intervals thereafter. RESULTS: Atrial pacing resulted in a marked decline in AF recurrences (p < 0.001). During dual-site pacing with an optimal drug regimen, there was no AF recurrence in any patient compared with five recurrences in 12 patients during single-site pacing (p = 0.03). The mean (+/-SD) arrhythmia-free interval before pacing (14 +/- 14 days) was prolonged with dual- (89 +/- 7 days, p < 0.0001) and single-site pacing (76 +/- 27 days, p < 0.0001). Symptomatic AF episodes showed a declining trend during dual- and single-site pacing compared with those during the preimplantation period (p = 0.10). Mean antiarrhythmic drug use for all classes declined from 4 +/- 1.9 drugs before implantation to 1.5 +/- 0.5 (p < 0.01) drugs after implantation. Twelve (80%) of 15 patients remained in atrial paced rhythm at 13 +/- 3 months. CONCLUSIONS: We conclude that multisite right atrial pacing is feasible, effective and safe for long-term application. Atrial pacing significantly prolongs arrhythmia-free intervals in patients with drug-refractory paroxysmal AF. Dual-site right atrial pacing may offer additional benefits and should be considered either as the primary mode or in patients unresponsive to single-site pacing.     

Systemic sclerosis with pulmonary involvement and right ventricular failure in a child

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.