Frequency and predictors of a high clinical response in patients with psoriasis on biological therapy in daily practice: results from the prospective, multicenter BioCAPTURE cohort.

BACKGROUND: It is important to assess which patients with psoriasis are more likely to achieve high clinical responses on biologics. OBJECTIVES: To assess the number of treatment episodes (TEs) that achieve a 100% improvement in Psoriasis Area and Severity Index (PASI 100), PASI 90 or PASI ≤ 5 at week 24 of biological treatment, and which baseline patient characteristics predict treatment response. METHODS: Data from patients with psoriasis treated with adalimumab, etanercept, infliximab or ustekinumab were extracted from a prospective cohort. TEs with high clinical responses were described. Uni- and multivariate regression analyses were performed with the generalized estimating equation method to elucidate which baseline patient characteristics were predictors for PASI 90 and PASI ≤ 5 at week 24. RESULTS: In total, 454 TEs were extracted (159 adalimumab; 193 etanercept; 19 infliximab; 83 ustekinumab) from 326 patients. At week 24, in 3%, 15% and 59% of TEs, respectively, PASI 100, PASI 90 and PASI ≤ 5 was reached. In TEs without a PASI 100 or PASI 90 response, PASI ≤ 5 was still achieved in 58% and 52%, respectively. Baseline PASI ≥ 10 was a strong predictor for achieving PASI 90; baseline PASI < 10 and a lower baseline body mass index (BMI) were significant predictors for PASI ≤ 5 at week 24. CONCLUSIONS: A limited number of patients achieved PASI 100 or PASI 90 at 24 weeks of biological treatment. Including an absolute PASI score in the assessment of psoriasis severity is important. Baseline BMI was an important, modifiable predictor for a high response.

Modified Limberg transposition flap in the treatment of pilonidal sinus disease.

BACKGROUND: Pilonidal sinus disease (PSD) is a common problem in surgical practice. Different non-surgical and surgical methods have been used for treating PSD. Flap techniques including the Limberg flap have become more popular in recent years. A modified Limberg flap was used to reduce the problems of skin maceration and recurrence associated with the conventional Limberg flap technique. The aim of this retrospective study was to assess the effectiveness of the modified Limberg flap technique for PSD. METHODS: Medical records of 94 patients with PSD who had been treated with a modified Limberg flap between December 2006 and 2009 were evaluated. The patients' age, sex, duration of preoperative symptoms, operative time, mean hospital stay, postoperative complications, wound infection rate, maceration rate and recurrence rate, time until return to work, time until sitting on the toilet without pain, hypoesthesia in the gluteal region, and satisfaction score were recorded during follow-up or at the last interview. Clinical data were obtained at the end of the 5th postoperative day and at 1, 3, 6, and 12 months following surgery. RESULTS: There were 83 male and 11 female patients. The mean operative time was 38.95 ± 6.77 min (range 30-67 min). All patients were followed up longer than 12 months, and the mean follow-up period was 30.97 ± 12.7 months (range 12-54 months). While wound dehiscence was observed in only one patient, we did not detect any case of flap necrosis. Two cases of seroma were observed. Wound infection was detected in 5 patients (5.3%). Surgical drainage was performed in 2 cases. Another 3 patients were treated with oral antibiotics. Maceration of the surgical incision site was detected in 8 patients (8.5%) who were all successfully treated with conservative measures. There were 4 patients (4.2%) with recurrence in this series. CONCLUSIONS: When compared with the available data on use of the conventional Limberg flap for PSD, our results suggest that use of the modified Limberg flap is associated with a lower maceration and recurrence rate, and greater patient satisfaction.

Two-year follow-up of a dose reduction strategy trial of biologics adalimumab, etanercept, and ustekinumab in psoriasis patients in daily practice.

BACKGROUND/OBJECTIVES: Tightly-controlled dose reduction was possible during 1 year in psoriasis patients on adalimumab, etanercept or ustekinumab with low disease activity (CONDOR trial). Extended observation is needed to ensure long-term effectiveness and safety of the strategy. With prolonged follow-up, we investigated the clinical effects and safety of the strategy, the proportion of patients with successful dose reduction, and assessed if patients with a disease flare regained remission. METHODS: Two-year follow up of a subgroup of patients previously included in a randomized pragmatic study comparing usual care (UC) with stepwise dose reduction (DR). Effectiveness (Psoriasis Area and Severity Index, PASI), Dermatology Life Quality Index (DLQI), adverse events, proportion of patients with successful DR and proportion of persistent disease flares were analyzed. RESULTS: DR leads temporarily to a slightly increased PASI groupwise, but on the long-term patients regained low PASI. DLQI scores remained stable during follow-up. No serious adverse events due to DR were reported. Forty-one percent of patients remained on a low dose up to 2 years. The number of persistent flares was low in DR and UC. CONCLUSIONS: The proposed dose reduction strategy is effective for a significant part of patients and remains safe up to 2 years of follow-up.

Unmet Personal Patient Needs in Psoriasis Patients with Low Disease Activity on Adalimumab, Etanercept or Ustekinumab.

BACKGROUND: Personal treatment goals have been systematically investigated in psoriasis patients with active but not in controlled disease. OBJECTIVES: To explore patient needs in psoriasis patients with controlled disease due to biologic therapy with adalimumab, etanercept or ustekinumab. METHODS: Treatment needs in patients on adalimumab, etanercept or ustekinumab with a stable low disease activity for ≥ 6 months and preferably a Psoriasis Area and Severity Index (PASI) < 5, were explored with the Patient Needs Questionnaire (PNQ). Goal importance was expressed as overall mean importance score, percentage of patients that reported a goal to be quite/very important, and per PNQ subscale. Data were analysed separately for treatment, gender, age group (< 50 vs. ≥ 50 years), biologic naivety and willingness to participate in a pragmatic dose-reduction strategy. RESULTS: Sixty-five patients were included. 'To be free of itching', 'to be healed of all skin defects' and 'to have confidence in the therapy' were rated quite/very important in 78.5% of the patients, followed by 'to have no fear the disease will progress' (75.4%) and 'to get better skin quickly' (75.4%). Goals related to the subscale 'confidence in healing' were still of high importance in controlled disease. Least importance was attributed towards social goals. For female patients, it was significantly more important than for males to 'feel less depressed' and 'be comfortable showing yourself more in public'. CONCLUSIONS: Psoriasis patients with controlled disease still report substantial treatment needs, with high importance ascribed to confidence in healing. To apply personalized medicine, treatment needs should be explored on an individual level.

In psoriasis patients, a large reduction in disease severity can lead to a significant improvement in health-related quality of life. In addition to quality-of-life measurements, individual treatment goals can be assessed to evaluate patients' preferences regarding their psoriasis treatment. As opposed to patients with more severe psoriasis, unmet treatment needs in psoriasis patients with stable, low disease activity have barely been reported. In this study, the personal treatment aims of patients with controlled disease due to treatment with adalimumab, etanercept or ustekinumab were explored using the Patient Needs Questionnaire. Sixty-five patients with sustained low disease activity for ≥ 6 months were included. We found that despite low disease activity, these patients still have substantial patient needs. Patients attributed the highest importance to goals on confidence in healing, in contrast to social goals, which were valued of least importance. For female patients, it was significantly more important to 'feel less depressed' and 'be comfortable showing yourself more in public' compared to male patients. Previous treatment with biologic therapy was not associated with an altered attitude towards specific treatment goals. Our population with low disease activity seemed to award a lower level of importance to all treatment goals compared to groups of patients with more severe psoriasis that have been described in literature. Since treatment goals differ per patient, individual treatment could be optimized by actively inquiring about the patient's personal treatment goals. Clinicians should be aware that even in patients with controlled disease, substantial personal treatment needs remain.

Cannabidiol protects keratinocyte cell membranes following exposure to UVB and hydrogen peroxide.

Keratinocytes, the major cell type of the epidermis, are particularly sensitive to environmental factors including exposure to sunlight and chemical agents. Since oxidative stress may arise as a result of these factors, compounds are actively sought that can act as protective agents. Recently, cannabidiol (CBD), a phytocannabinoid found in Cannabis Sativa L., has gained increased interest due to its anti-inflammatory and antioxidant properties, and absence of psychoactive effects. This prompted us to analyze the protective effects of CBD on keratinocytes exposed to UVB irradiation and hydrogen peroxide. Here we show, using liquid chromatography mass spectrometry, that CBD was able to penetrate keratinocytes, and accumulated within the cellular membrane. CBD reduced redox balance shift, towards oxidative stress, caused by exposure UVB/hydrogen peroxide, estimated by superoxide anion radical generation and total antioxidant status and consequently lipid peroxidation level. CBD was found to protect keratinocytes by preventing changes in the composition of the cellular membrane, associated with UVB/hydrogen peroxide damages which included reduced polyunsaturated fatty acid levels, increased sialic acid and lipid peroxidation products (malondialdehyde and 8-isoprostanes) levels. This maintains cell membranes integrity and prevents the release of lactate dehydrogenase. In addition, CBD prevented UVB/hydrogen peroxide-induced reduction of keratinocyte size and zeta potential, and also decreased activity of ATP-binding cassette membrane transporters. Together, these findings suggest that CBD could be a potential protective agent for keratinocytes against the harmful effects of irradiation and chemical environmental factors that cause oxidative stress.

First pulmonary valve replacement with Freedom Solo stentless valve in an adolescent. A case report.

Tetralogy of Fallot (TOF) is a common form of cyanotic heart disease. Complete surgical correction in younger age group offers good long-term results with reasonable morbidity and improved prognosis in patients with TOF. However, following corrective surgery pulmonary valve replacement (PVR) might be required for residual pulmonary regurgitation in order to avoid irreversible right ventricular remodeling. Otherwise, residual uncorrected pulmonary regurgitation may lead to right ventricular dilatation, impaired biventricular function, ventricular arrhythmias and limited exercise capacity. We report the first case of Freedom Solo stentless valve (Sorin Group, Saluggia, Italy) implantation in the pulmonary position in an adolescent with severe pulmonary insufficiency 12 years after the repair of TOF. Pericardial stentless valves may be an alternative choice for pulmonary valve replacement to improve right ventricular contractile recovery and remodeling after PVR and may have impact on long-term survival.

Dispersion of QT and QTc interval in healthy children, and effects of sinus arrhythmia on QT dispersion.

OBJECTIVE: To determine the normal values of QT and QTc dispersion and the effects of sinus arrhythmia on QT dispersion in healthy children. PATIENTS AND SETTING: The study was carried out in a university hospital on 372 local schoolchildren (200 male, 172 female), aged seven to 18 years. METHODS: The QT and preceding RR intervals of at least one sinus beat were measured manually in a range of nine to 12 leads on standard 12 lead surface ECGs. The corrected QT interval was computed by the method of Bazett. Dispersion of QT and QTc were defined as (1) the difference between the maximum and minimum QT and QTc intervals occurring in any of the 12 leads (QTD and QTcD), (2) the standard deviation of the QT and QTc interval in the measurable leads (QT-SD and QTc-SD). RESULTS: There was no significant difference in QT, QTc, and RR dispersion between girls and boys. Overall 53% of children had sinus arrhythmia. Although QTD and QT-SD were not affected by sinus arrhythmia, both QTcD and QTc-SD were significantly greater in children with sinus arrhythmia than in those without (QTcD: 52.9 (17.4) v 40.9 (13.1); QTc-SD: 17.5 (5.9) v 13.2 (4.0); p < 0.001). CONCLUSIONS: As calculation of QTc dispersion is affected by sinus arrhythmia, which is common in childhood, we suggest that QT dispersion should not be corrected for heart rate in children.

Plasma endothelin-1 levels in patients with left-to-right shunt with or without pulmonary hypertension.

The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmonary hypertension (PH) secondary to congenital heart disease with left-to-right shunt. Twenty-three children (12 male, 11 female) aged 0.58-13 years were enrolled the study. Blood samples were drawn from superior vena cava, right atrium, right ventricle, pulmonary artery and pulmonary wedge or pulmonary vein during cardiac catheterization. Plasma ET-1 levels were assayed by ELISA. Patients were divided into two groups according to the presence or absence of PH. Plasma ET-1 levels of the study group were compared to the peripheral venous and arterial ET-1 levels of 11 healthy infants and children (aged 0.75-13 years). Plasma ET-1 levels in patients with left-to-right shunt were found significantly higher than those of controls. However, plasma ET-1 levels were similar between the two groups of the patients. Pulmonary venous ET-1 levels were higher than the levels of superior vena cava, this suggested an increased production of ET-1 in pulmonary vascular bed in patients with PH. No correlations were found between plasma ET-1 levels and pulmonary arterial pressure, pulmonary vascular resistance and pulmonary blood flow in the patients. Plasma ET-1 levels of the patients with left-to-right shunt were increased independently from pulmonary arterial pressure and pulmonary vascular resistance. This increase was related to the production of ET-1 in pulmonary vascular bed in patients with PH. ET-1 could not be found to be directly related to the development of PH in the patients with left-to-right shunt.

Quantitation of Doppler color flow jet areas for mitral regurgitation in children: angiographic correlation.

Eighteen patients with chronic isolated rheumatic mitral regurgitation aged between 7 and 19 years (mean age +/-SD, 12.69+/-3.47 years) were analyzed with color Doppler imaging. Sixteen patients were performed cardiac catheterization within 24 h. Jets were classified as eccentric and central. Regurgitant jet area and its ratio to left atrial area and body surface area were measured by Doppler color flow imaging. Regurgitant volume and regurgitant fractions were calculated with angiography. There was a good correlation between regurgitant jet area and angiographic grade of mitral regurgitation (P<0.01). The correlation between regurgitant jet area/left atrial area ratios and angiographic grade of mitral regurgitation was limited (P<0.01). There was excellent correlation between regurgitant jet area/body surface area and angiographic regurgitant fraction (r = 0.85; P<0.001). There was also a good correlation between regurgitant jet area and regurgitant fraction (r = 0.82; P<0.001). However, the relation of regurgitant jet area/left atrial area to regurgitant fraction was weak (r = 0.72; P<0.01). In conclusion, the measurement of regurgitant fraction and its ratios to left atrial area and body surface area by color Doppler flow imaging can predict the angiographic severity in children who have even eccentric regurgitant jets.

QT/corrected QT (QTc) intervals and QT/QTc dispersions in children with chronic renal failure.

UNLABELLED: We aimed to examine QT/corrected QT (QTc) intervals, QT/QTc dispersions (QTD/QTcD) and also the effect of different clinical and laboratory variables on these parameters in children with chronic renal failure. Serum biochemistry, 12-lead electrocardiogram, telecardiogram, and echocardiography were performed in 50 children with chronic renal failure (23 female and 27 male; aged 12.3+/-3.6 years, range 5 to 20 years). None of them had symptoms related to arrhythmias. When compared with a control group (372 children, aged 7 to 18 years, mean 12.4+/-2.6) patients with chronic renal failure had greater QT/QTc intervals and QT/QTc dispersion values (Patient: QT = 360.9+/-53.3; QTc = 438.5+/-33.2; QTD = 42.4+/-20.8; QTcD = 57.5+/-23.8; CONTROL: QT = 325.9+/-24.1; QTc = 398.7+/-19.7; QTD = 29.9+/-10.2; QTcD = 47.3+/-16.6; P<0.01). QT, QTc, and QTcD values were significantly greater in patients who had renal failure duration longer than 2 years. Patients who had impaired left ventricular systolic function on echocardiogram had greater QTc, QTD, and QTcD values. It was found that sex, cardiomegaly on chest X-ray, and left ventricular hypertrophy on echocardiogram were not related to these parameters. It is concluded that, impaired cardiac systolic function and longer renal failure duration are related to an increase in QT, QTc, QTD, and QTcD values and hence these variables may be risk factors for ventricular arrhythmias in uremic patients.

Impact of dialysis adequacy on cardiac function in pediatric CAPD patients.

BACKGROUND: Left ventricular hypertrophy is a major cause of morbidity and mortality among patients with chronic renal failure. Uremia-related risk factors play a fundamental role in its occurrence, thus better prognosis and prolonged survival can be attained by successful dialytic therapies. OBJECTIVE: To investigate whether dialysis adequacy has a beneficial effect on cardiac structure and function in children receiving continuous ambulatory peritoneal dialysis (CAPD). DESIGN: Cross-sectional study in the Pediatric Peritoneal Dialysis Unit of a university hospital. PATIENTS: Eighteen children, aged 13.3 +/- 2.8 years, being treated with CAPD, and 20 healthy age- and sex-matched control subjects were enrolled in this study. MAIN OUTCOME MEASURES: Echocardiographic evaluation was performed in all subjects. Dialysis adequacy indices [weekly urea (Kt/V) and creatinine clearance (TCCr)] were calculated in the dialysis group. RESULTS: Interventricular septal thickness, left ventricular (LV) posterior wall thickness, LV mass index (LVMI), and LV end systolic and diastolic dimensions were all found to be significantly higher in the CAPD group compared to the control subjects (p < 0.01). Ejection fraction and fractional shortening of the LV were not significantly different between the two groups. Mean Kt/V was 2.02 +/- 0.71 and mean TCCr was 58 +/- 33 L/wk/1.73 m2. There were significant negative correlations between dialysis adequacy indices and LV end systolic and diastolic dimensions (p < 0.05 and p < 0.001). Ejection fraction and fractional shortening were positively correlated with Kt/V (p < 0.01). Systolic and diastolic blood pressures were positively correlated with LVMI (r= 0.501 and r = 0.523). Significant inverse correlations between mean arterial pressure and both Kt/V and TCCr (r = -0.555 and r = -0.520) were detected. CONCLUSION: These data clearly document that cardiac structure and function are remarkably influenced by the uremic state and dialysis therapy in pediatric CAPD patients. The close relationships between echocardiographic findings and dialysis adequacy indices suggest that adequate dialysis has a beneficial effect on cardiac function via effective removal of toxic substances.

Microleakage and SEM evaluation of fissure sealants placed by use of self-etching priming agents.

OBJECTIVES: This study evaluated the microleakage and internal seal of fissure sealants placed by use of self-etching priming agents in comparison to phosphoric acid etching of enamel. METHODS: Seventy-two caries-free extracted human molars were divided into six groups with 12 teeth each. Occlusal surfaces were cleansed by either pumicing (Groups I, III, V) or by 15-s air-abrasion treatment with 25 microm aluminum oxide particles (Groups II, IV, VI). Fissures were sealed with the self-etching priming systems, Clearfil Liner Bond 2 (Groups I, II) or Resulcin AquaPrime (Groups III, IV). In Groups V and VI, sealants were placed after phosphoric acid etching. Half of the teeth in each group were thermocycled. After staining with 0.5% methylene blue, the teeth were sectioned for evaluation of microleakage. Internal adaptation of the fissure sealants was analyzed by SEM on replicas of cross sections. RESULTS: Independent of the methods used for cleansing of the occlusal surfaces, fissure sealants in Groups I and II showed significantly more microleakage and less sufficient internal seal as compared to sealants placed in Groups III to VI. Sealants placed by Resulcin AquaPrime (Groups III, IV) leaked significantly more than sealants applied after phosphoric acid etching (Groups V, VI) of the enamel. However, statistical analysis (H-test) did not reveal significant differences concerning the internal adaptation of sealants placed in Groups III, IV, V and VI. CONCLUSIONS: Concerning the microleakage data, use of the self-etching bonding systems, Clearfil Liner Bond 2 and Resulcin AquaPrime, cannot be recommended for fissure sealing, since the sealing ability is less effective as compared to the conventional acid-etching technique.

Mitral valve and left ventricular thrombi in an infant with acquired protein C deficiency. A case report.

The authors present an infant with left ventricular and mitral valve thrombi diagnosed by cross-sectional echocardiography. Thrombosis was due to acquired transient protein C deficiency, which was caused by impaired liver function due to sepsis. Because the thombi were very large and mobile, urgent surgery was performed. Eight weeks later, the patient's protein C level returned to normal ranges. The authors suggest that in all cases with intracardiac thrombosis, protein C deficiency should be investigated.

Echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract: possible role in the pathogenesis of discrete subaortic stenosis.

Although the clinical features and natural course of discrete subaortic stenosis (DSS) are well defined, the etiology remains speculative. The purpose of this study was to identify the echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract associated with DSS in children and to determine whether these variations have a role in the pathogenesis of DSS. The aortoseptal angle (ASA), mitral-aortic valve separation (MAS), and the size of the aortic annulus were determined in two groups of children. Group 1 comprised 11 patients with isolated DSS, who were compared with an age- and body surface area- (BSA) matched healthy children (Group 1A, n: 20). Group 2 comprised 10 patients with DSS and ventricular septal defect (VSD). Group 2 was compared with an age- and BSA-matched patients with isolated perimembranous VSD (Group 2A, n: 22). Measurements were carried out from previously recorded echocardiographic studies. The ASA was steeper (119.3 +/- 6.1 degrees vs 137.5 +/- 5.6 degrees , p < 0.001), and the MAS was wider (6.1 +/- 1.6 vs 3.2 +/- 0.7 mm, p < 0.001) in patients with isolated DSS than in healthy control subjects. Similar differences were found between patients in Group 2 and Group 2A; the ASA was steeper (122.2 +/- 6.5 degrees vs 141.3 +/- 5.0 degrees, p < 0.001), and the MAS was wider (5.8 +/- 1.5 vs 3.8 +/- 1.1 mm, p < 0.001). The size of the aortic annulus was not different among the four study groups. Although the MAS was significantly wider in patients with DSS, there was significant overlap in MAS between patients and controls. However, if an ASA < or = 130 degrees was chosen as a predictive variable, it was found to be a highly sensitive, specific, and positive predictive marker for the development of DSS. This study demonstrates that DSS is associated with a steeper ASA, and a wider MAS, in patients with or without associated VSD. These morphologic abnormalities, especially a steeper ASA, may be risk factors for the development of DSS.

Relation of mass/volume ratio to ECG abnormalities and symptoms in children with aortic stenosis/insufficiency.

In this study, to determine whether symptoms and ECG abnormalities relate to left ventricular (LV) mass, volume, and mass/volume (M/V) ratio in children with aortic stenosis (AS) and/or insufficiency (AI), the authors examined 23 patients with echocardiography. LV volumes and mass were calculated with echocardiography. Also, the peak and mean gradients across the aortic valve and left ventricular meridional wall stress (ESWS) were determined by use of echo Doppler techniques. Fourteen patients (mean age 5.84+/-3.49 years) had AS alone. Of these, 14 had symptoms and seven had abnormal-appearing ECGs. Nine patients (mean age 6.91+/-4.35 years) had AS/AI. Of these nine, four had symptoms and two had ECG abnormalities. The authors observed that the incidences of symptoms and ECG abnormalities were higher in the patients with AS than in those with AS/AI. There was no significant correlation between symptoms and ECG abnormalities with peak gradient, mean gradient, valve area, LV mass, volume, and mass/volume ratio in patients with AS/AI. However, significant correlation was found between symptoms and ECG abnormalities and ESWS in AS/AI patients. In conclusion, ECG abnormalities and symptoms do not always indicate the severity of AS and AS/AI.

Recurrent pericardial effusion due to gunshot wound of the heart in a hemodynamically stable child--a case report.

A 12-year-old girl presented with recurrent pericardial effusion due to firearm pellet injury to the left ventricle. The pellet was localized by two-dimensional echocardiography within the left ventricular apical wall. Since the patient was asymptomatic, left ventriculotomy was avoided to extract the pellet and only pericardial tube drainage was carried out. A slightly elevated blood lead level of the patient was alarming for potential subsequent lead poisoning due to retained pellets.

Congenital isolated apical ventricular septal defects.

The diagnosis of apical ventricular septal defects (VSDs) is difficult and the ratio of spontaneous closure is not well known. At the Ankara University Pediatric Cardiology Department, between the years 1992 and 1997, 33 patients were identified by color-flow Doppler (CFD) imaging as having a small defect at the apical portion of the ventricular septum. The age range was 1 day to 13 years. All patients were asymptomatic with normal telecardiograms and electrocardiograms. There were grade 2-3/6, pansystolic murmurs at the apex or closer to the apex in 19 patients. In 30 patients, narrow and short colored jets were seen at the apex of the heart, distal to the moderator band. The jets were wide in three patients. These defects were difficult to image and required specific angulation of the transducer. Of the 23 patients who had follow-up examinations, at 3 months to 3.5 years, there was spontaneous closure in ten (43.5%). To the authors' knowledge, this report is the largest series of isolated apical trabecular ventricular septal defects (t-VSDs). In their experience, color-flow imaging is more sensitive in detecting a small apical VSD. They conclude that although the closure of apical VSD is not the rule, the prognosis is excellent.

Congenital complete atrioventricular block in an infant with long QT syndrome.

A three months old patient with congenital complete atrioventricular block and prolonged QT interval syndrome is presented. Before admission she had had two syncopal attacks. During hospitalization she had an episode of torsade de pointes which was followed by ventricular fibrillation. After a successful cardiopulmonary resuscitation, a permanent epicardial ventricular pacemaker was implanted and she was discharged on propranolol therapy. She is now free of symptoms and doing well, 16 months after discharge.