Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.

BACKGROUND: Congenital absence of the pericardium (CAP) is a rare clinical entity. METHODS: We identified from the two hospital databases all patients with isolated CAP, reviewed their data, and invited them for prospective clinical evaluation with electrocardiography, chest x-ray findings (CXR), echocardiography, and magnetic resonance imaging (MRI). RESULTS: Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely nonexertional (9), and heart murmur with an abnormal CXR (1). Three patients had partial and 7 had complete CAP (all 7 had marked lateral displacement of the cardiac apex). CXR combined with MRI were key to establishing the diagnosis; a "tongue" of lung tissue interposing between the main pulmonary artery and aorta was the most consistent diagnostic feature. Four patients underwent pericardioplasty, 3 for debilitating symptoms and 1 for left atrial appendage herniation, followed by improvement or resolution of symptoms. At a mean of 10.5 years from presentation all patients were alive. No complications were seen in the nonsurgical group. CONCLUSIONS: Isolated CAP has a common presentation pattern with periodic stabbing chest pain mimicking coronary artery disease. CXR and MRI are required for definitive diagnosis. Symptomatic patients with the complete form may benefit from pericardioplasty.

Cardiac tamponade and death from intrapericardial rupture [corrected] of sinus of Valsalva aneurysm.

A 35-year-old woman presented with dyspnea and chest pain. She had a large aneurysm of the non-coronary sinus of Valsalva. Before her scheduled urgent surgery, the patient collapsed and died of cardiac tamponade secondary to intrapericardial rupture of the aneurysm. We would advocate urgent repair of this type of lesion to prevent such an outcome. We are aware of no other specific reports addressing extracardiac rupture of non-coronary cusp aneurysms [corrected].

Use of complementary and alternative treatment for allergic contact dermatitis.

BACKGROUND: Previous studies show that use of complementary and alternative medicine (CAM) is frequent among dermatological patients in general and that the use of CAM is linked to disease severity and duration. OBJECTIVES: To investigate factors influencing the use of CAM and visits to alternative practitioners among patients with allergic contact dermatitis. METHODS: The study was a postal questionnaire survey among 485 patients with allergic contact dermatitis to fragrances and preservatives in consumer products. The questionnaire included questions about use of conventional treatment, use of CAM and visits to alternative therapists. It also included questions about eczema severity and duration as well as social factors. The patients were recruited from one dermatological clinic in the capital city area and two clinics in smaller cities. RESULTS: The response rate was 79%. Forty per cent of respondents were users of CAM, predominantly in combination with conventional treatment, and 29% had visited an alternative practitioner. Women were found to be significantly more frequent users of CAM and visitors to alternative practitioners than men, and patients from urban/rural districts reported more frequent use of CAM than patients from the capital city area. Frequent eczema eruptions, hand eczema, long duration of disease and work-related problems were positively associated with use of CAM and/or visits to alternative practitioners. CONCLUSIONS: The typical CAM user is female, lives in a rural district or town (not the capital area), has long disease duration and often work-related problems. Overall, the use of CAM is not an alternative to conventional treatment, but is a supplement.

Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology.

OBJECTIVE: To compare the relative merits of cavopulmonary or aortopulmonary shunts, or both, as definitive non-Fontan palliations for patients with single ventricle physiology. DESIGN: Clinical data, ECG, echocardiographic data, surgical records, and available postmortem material were reviewed in all patients with single ventricle physiology identified from the University of Toronto Congenital Cardiac Centre for Adults (UTCCCA) database who had not undergone a Fontan operation. Current status of patients was assessed from clinic reviews and patient contact. Two groups of patients were identified: those with cavopulmonary shunt (group 1, n = 35); and those with aortopulmonary shunt(s) only (group 2, n = 15). RESULTS: 50 adults (21 male/29 female) who underwent the last palliation at a median age of 11 years (range 1 day to 53 years) were identified. During a mean (SD) follow up of 13.0 (6.2) years at the UTCCCA, 19 patients died. Survival is 89.4% and 51.9% at 10 and 20 years, respectively, from the time patients were first seen at UTCCCA, with no differences between the groups. Most recent New York Heart Association (NYHA) classification was I-II in 21 patients, III in 25, and IV in four patients; mean haemoglobin was 190 (28) g/l, and oxygen saturation was 82 (4)%, with no group differences. Arrhythmia developed in 25 patients (atrial flutter/fibrillation in 20 and/or sustained ventricular tachycardia in 11). Atrial flutter/fibrillation was more common in patients in group 2, who also showed a greater decline in ventricular function with time. Age at last palliation, cardiothoracic ratio, and inclusion in group 2 were predictive of atrial flutter/fibrillation, poor ventricular function predictive of ventricular tachycardia, NYHA class > III, and prior ventricular tachycardia predictive of death. CONCLUSIONS: Cavopulmonary or aortopulmonary shunts, or both, provide sustained palliation for selected patients with single ventricle physiology. Survival for both compares favourably with published Fontan series. Compared to aortopulmonary shunts, cavopulmonary shunts convey a beneficial long term effect on ventricular function. Arrhythmia is a major cause of late morbidity in these patients, relating to both ventricular dysfunction and death. Onset of sustained ventricular tachycardia is an ominous sign.