Coronary Artery Bypass Grafting and Percutaneous Coronary Intervention after Kawasaki Disease: The Pediatric Canadian Series.

Coronary artery (CA) aneurysms are serious complications of Kawasaki disease (KD) responsible for ischemic events. Percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG) are reported with limited data on indications and comparative efficacy. Retrospective multicenter comparison of CA intervention following KD is performed in this study. Twenty two cases were available from 5 centers, of whom 11 underwent CABG, 10 PCI and 1 systemic thrombolysis. Age at intervention (8.3 ± 3.9 vs 11.3 ± 4.9 years, p = 0.14) and interval from diagnosis (5.6 ± 4.1 vs 6.5 ± 4.7 years, p = 0.64) were similar between CABG and PCI. Interventions were based on angiography in 15 patients or cardiac event in 7, with no difference between CABG and PCI (p = 0.24). Patients with CABG were more likely to undergo multivessel intervention (73 vs 10 %, p = 0.006). None of the patients needed reintervention after CABG, compared to 6 after PCI and 1 after systemic thrombolysis (p = 0.004). Signs of ischemia on stress testing or MIBI were present in 15 patients before intervention and persisted in 9 patients following last intervention, in a significantly higher proportion after CABG than PCI (80 vs 17 %, p = 0.01). In this series, CABG, which mostly involved multivessel intervention, was superior to PCI. Nevertheless, larger-scale studies may help define patient selection criteria for a beneficial PCI approach.

Deaths in Rotorua's geothermal hot pools: hydrogen sulphide poisoning.

In late 2007 and early 2008 two gentlemen were found dead in, or near to, enclosed hot pools fed with Rotorua's geothermal waters. Amidst much publicity the Coroner has ruled that the deaths were related to hydrogen sulphide poisoning. Following post mortem examinations, blood and urine samples were frozen and sent to the Toxicology Unit of ESR. These were then stored frozen until analysis. Hydrogen sulphide (H(2)S) is a potentially deadly gas at elevated levels, but is rapidly eliminated from the body and is unstable post mortem. Thiosulphate is a marker for the exposure to H(2)S, and as it is stable post mortem the samples were analysed to determine the thiosulphate levels present. The urine thiosulphate levels detected were above those seen in the urine samples measured from the only previous study of people exposed to the Rotorua thermal area and the blood levels were similar to literature values from fatalities exposed in workplaces such as sewage treatment plants.

Counselling the terminally ill - Can we prepare for death?

The family medical practitioner is a key player when care of the patient becomes palliative. The practitioner's ability to be present to the psychological; spiritual and social needs - in addition to the physical -gives reassurance and affirmation of life up to the point of death. Counselling in the medical field and the approach to opening up a conversation with a patient or family member is discussed

A national network for the tele-education of Canadian residents in pediatric cardiology.

A trial of 11 video-conferenced teaching sessions for residents in pediatric cardiology was performed by the 7 training programs in Canada in order to share expertise in specialized areas, to expose trainees to educational telemedicine, and to acquaint residents with other programs and personnel. Topics included cardiac pathology, arrhythmias, magnetic resonance imaging, fetal physiology, pulmonary hypertension, and cardiomyopathy. The sessions were evaluated by 93 residents by questionnaire for content and technology. Session content was highly rated. Videoconference picture quality was highly rated, but sound quality and visual aids were rated as neutral or unsatisfactory by a significant minority, related to problems with several early sessions, subsequently corrected. 60% of respondents rated the videoconferences as good as live presentations. Presenters were generally satisfied although they required some adjustments to videoconferencing. The average cost per session was $700 Canadian. Videoconferencing of resident educational sessions was generally well accepted by most presenters and residents, and the trial has formed the basis for a national network. Adequate organizational time, and careful attention to audiovisual needs, are most important. Videoconference guidelines are suggested for presenters based on this experience.

Acute rheumatic fever and poststreptococcal reactive arthritis: diagnostic and treatment practices of pediatric subspecialists in Canada.

OBJECTIVE: We conducted a survey of pediatric specialists in rheumatology, cardiology, and infectious diseases to ascertain present Canadian clinical practice with respect to diagnosis and treatment of acute rheumatic fever (ARF) and poststreptococcal reactive arthritis (PSReA), and to determine what variables influence the decision for or against prophylaxis in these cases. METHODS: A questionnaire comprising 6 clinical case scenarios of acute arthritis occurring after recent streptococcal pharyngitis was sent to members of the Canadian Pediatric Rheumatology Association, and to heads of divisions of pediatric cardiology and pediatric infectious diseases at the 16 university affiliated centers across Canada. RESULTS: There is considerable variability with respect to diagnosis in cases of ReA following group A streptococcal (GAS) infection both within and across specialties. There is extensive variability regarding the decision to provide prophylaxis in cases designated as ARF or PSReA. Findings indicated that physicians are most comfortable prescribing antibiotic prophylaxis in the presence of clear cardiac risk and are less inclined to such intervention for patients diagnosed with PSReA. When prophylaxis was recommended for cases of PSReA, the majority of respondents prescribed longer term courses of antibiotics. CONCLUSION: The lack of observed consistency in diagnosis and treatment in cases of reactive arthritis post-GAS infection likely reflects the lack of universally accepted criteria for diagnosis of PSReA and insufficient longterm data regarding carditis risk within this population. There is a need for clear definitions and treatment guidelines to allow greater consistency in clinical practice across pediatric specialties.

A multicentre international study of sedation for uncontrolled symptoms in terminally ill patients.

The issue of symptom management at the end of life and the need to use sedation has become a controversial topic. This debate has been intensified by the suggestion that sedation may correlate with 'slow euthanasia'. The need to have more facts and less anecdote was a motivating factor in this multicentre study. Four palliative care programmes in Israel, South Africa, and Spain agreed to participate. The target population was palliative care patients in an inpatient setting. Information was collected on demographics, major symptom distress, and intent and need to use sedatives in the last week of life. Further data on level of consciousness, adequacy of symptom control, and opioids and psychotropic agents used during the final week of life was recorded. As the final week of life can be difficult to predict, treating physicians were asked to complete the data at the time of death. The data available for analysis included 100 patients each from Israel and Madrid, 94 patients from Durban, and 93 patients from Cape Town. More than 90% of patients required medical management for pain, dyspnoea, delirium and/or nausea in the final week of life. The intent to sedate varied from 15% to 36%, with delirium being the most common problem requiring sedation. There were variations in the need to sedate patients for dyspnoea, and existential and family distress. Midazolam was the most common medication prescribed to achieve sedation. The diversity in symptom distress, intent to sedate and use of sedatives, provides further knowledge in characterizing and describing the use of deliberate pharmacological sedation for problematic symptoms at the end of life. The international nature of the patient population studied enhances our understanding of potential differences in definition of symptom issues, variation of clinical practice, and cultural and psychosocial influences.

Natural history and surgical outcomes for isolated discrete subaortic stenosis in children.

OBJECTIVE: To document the natural history and surgical outcomes for discrete subaortic stenosis in children. DESIGN: Retrospective review. SETTING: Tertiary care paediatric cardiology centres. PATIENTS: 92 children diagnosed between 1985 and 1998. MAIN OUTCOME MEASURES: Echocardiographic left ventricular outflow gradient (echograd), and aortic insufficiency (AI). RESULTS: The mean (SEM) age at diagnosis was 5.3 (0.4) years; the mean echograd was 30 (2) mm Hg, with AI in 22% (19/87) of patients. The echograd and incidence of AI increased to 35 (3) mm Hg and 53% (36/68) (p < 0.05) 3.6 (0.3) years later. The echograd at diagnosis predicted echograd progression and appearance of AI. 42 patients underwent surgery 2.2 (0.4) years after diagnosis. Preoperatively echograd and AI incidence increased to 58 (6) mm Hg and 76% (19/25) (p < 0.05). The echograd was 26 (4) mm Hg 3.7 (0.4) years postoperatively, with AI in 82% (31/38) of patients. Surgical morbidities included complete heart block, need for prosthetic valves, and iatrogenic ventricular septal defects. Eight patients underwent reoperation for recurrent subaortic stenosis. The age at diagnosis of 44 patients followed medically and 42 patients operated on did not differ (5.5 (0.6) v 5. 0 (0.6) years, p < 0.05). However, the echograd at diagnosis in the former was less (21 (2) v 40 (5) mm Hg, p < 0.05) and did not increase (23 (2) mm Hg) despite longer follow up (4.1 (0.4) v 2.2 (0. 4) years, p < 0.05). The incidence of AI at diagnosis and at last medical follow up was also less (14% (6/44) v 34% (13/38); 40% (17/43) v 76% (19/25), p < 0.05). CONCLUSIONS: Many children with mild subaortic stenosis exhibit little progression of obstruction or AI and need not undergo immediate surgery. Others with more severe subaortic stenosis may progress precipitously and will benefit from early resection despite risks of surgical morbidity and recurrence.

Role of the toxic neutrophil count in the early diagnosis of Kawasaki disease.

OBJECTIVE: To determine whether the toxic neutrophil count (TNC), defined as the sum of the number of peripheral blood neutrophils with vacuoles plus the number with toxic granulations per 100 neutrophils examined, can be used as an aid to early diagnosis of Kawasaki disease (KD). METHODS: Prospective evaluation at a tertiary care pediatric hospital of 56 acutely febrile children with at least one other clinical criterion for KD. Clinical characteristics and TNC were compared for 3 groups of patients: those with (1) definite KD, (2) probable KD, and (3) unlikely KD. The sensitivity and specificity of the TNC at various cutoff points was determined. RESULTS: We evaluated 56 patients (Group 1, N=27; Group 2, N=4; Group 3, N=25). Mean TNC (TNC/mm3) were higher in the patients with definite KD compared to patients with unlikely KD (38 vs 24; p=0.037). At a TNC cutoff of > or =70, the test had a specificity of 100%, but a sensitivity of only 18%. The likelihood ratio (the number of times more likely this TNC result is to be found in KD versus non-KD subjects) was 8.89. At a cutoff of > or =10, the test had a high sensitivity of 92% and specificity of 38%. CONCLUSION: No laboratory test replaced the need for careful clinical evaluation in cases of suspected KD. The TNC may be a useful adjunct to the clinical assessment of children with KD, particularly at the extremes of measurement.

Atrial and venous thrombosis secondary to septic arthritis of the sacroiliac joint in a child with hereditary protein C deficiency.

Septic arthritis and osteomyelitis in children is seldom accompanied by calf vein thrombosis and rarely by atrial thrombosis. We report the case of an 11-year, 5-month-old boy with septic arthritis and osteomyelitis of the sacroiliac region who developed deep venous thrombosis, in addition to life-threatening right atrial thrombosis. After an intensive hematologic investigation, a hereditary protein C deficiency was revealed. The association of venous thrombosis with septic arthritis or osteomyelitis should raise the possibility of the presence of protein C deficiency.

Cerebral blood flow velocity during occlusive manipulation of patent ductus arteriosus in children.

Patent ductus arteriosus (PDA) with left-to-right shunting modifies the Doppler flow pattern of the intracranial circulation. The ability of increases in cerebral blood flow velocity (CBFV) to predict shunt resolution during PDA occlusion was evaluated. A 2 MHz transcranial Doppler (TCD) monitored diastolic and mean CBFV, plus the systolic/mean CBFV ratio in the middle cerebral artery from before (baseline) to immediately after PDA occlusion. Shunt resolution was verified by echocardiography and/or angiography. A minimum of 40% increase in diastolic-CBFV from baseline was considered successful resolution. Patients were age-stratified into group I (< 15 months; n = 23) and group II (> 15 months; n = 10). Thirty-three children were studied (age, 0.1 to 109 months) during surgical (n = 22) or coil occlusions (n = 11). Transcranial Doppler successfully identified shunt resolution in 78% of cases in group I, as compared to 0% in group II (p < 0.01). Identification rate decreased from 79% in cases of minimum ductal diameter of 3 mm (n = 19) to 21% in smaller ductuses (n = 14) (p < 0.01). Body weight and left-atrium size (p = 0.004) in group I and PDA diameter in group II (p = 0.02), were the only preoperative ductal parameters associated with diastolic-CBFV changes after ductus occlusion. Transcranial Doppler detects shunt resolution in infants with moderate to large PDAs.

Aortic cusp prolapse in ventricular septal defect and its association with aortic regurgitation--appropriate timing of surgical repair and outcomes.

OBJECTIVES: To determine the appropriate indications and timing for surgery in children with either a perimembranous or a subarterial type of ventricular septal defect (VSD) associated with aortic cusp prolapse. DESIGN: Retrospective review of children with VSD and associated aortic cusp prolapse with or without aortic regurgitation. This review was based on data obtained from clinical findings, two-dimensional echocardiography, cardiac catheterization and angiocardiography. SETTING: Tertiary health care facility with two-dimensional and colour Doppler echocardiographic and cardiac surgery facilities, and a catheterization laboratory. PATIENTS: Forty-eight patients were found to have perimembranous or subarterial VSDs in association with aortic cusp prolapse with or without aortic regurgitation. INTERVENTIONS: All 48 patients had high resolution two-dimensional and colour Doppler echocardiography. Of the 19 patients who underwent surgical closure of their VSD, five also had an aortic valvuloplasty and one had an aortic valve replacement. Cardiac catheterization was performed in 16 of the 19 surgical patients and 12 of the 29 nonsurgical patients. MEASUREMENTS AND MAIN RESULTS: Annual clinical and echocardiographic assessments in the nonsurgical group did not demonstrate increasing aortic insufficiency. Two children in the nonsurgical group showed spontaneous resolution of aortic insufficiency. In the surgical group, four children with VSD and clinical aortic insufficiency had surgery at less than five years of age; two were found to be regurgitant-free, one had trivial clinical aortic insufficiency and the other had echocardiography-only insufficiency. Of the seven surgical patients older than five years with VSD and clinical aortic insufficiency, four were found to be regurgitant-free, one had echocardiography-only regurgitation and two were unchanged. Two children undergoing surgery with VSD and no aortic insufficiency had postoperative echocardiography-only regurgitation, presumably related to cusp deformity from presurgical prolapse. Children with large VSDs with or without aortic cusp prolapse required surgery for indications of shunt size and pulmonary resistance. CONCLUSIONS: For children with small perimembranous VSDs and cusp prolapse, surgery is indicated only if there is clinical evidence of aortic regurgitation and progressive left ventricular enlargement.

Transoesophageal echocardiographic assessment of obstruction to the pulmonary venous pathway in children with Mustard or Senning repair.

The morphology and mechanism of obstruction to the pulmonary venous pathway in patients following either Mustard or Senning repair of complete transposition was assessed using transoesophageal echocardiography. Seven patients underwent catheterization and complete transoesophageal study in both transverse and longitudinal planes, followed by balloon dilation of the obstructed venous pathway in five of seven under transoesophageal echocardiography guidance. A complete scan of both systemic and venous pathway was obtained in all patients. Four patients with a Mustard repair were found to have a 'tubular' baffle, with stenosis resulting from a discrete wedge of tissue arising from the atrial free wall in association with fibrous adhesions to the baffle. In the three patients with a Senning repair the intra-atrial baffle showed a characteristic 'peaked' appearance, with stenosis of the venous pathway stenosis related directly to contracture of the patch used to augment the atrial free wall. The mechanism of obstruction appears to be inherent to the different surgical techniques. Indwelling transoesophageal echocardiography provided immediate haemodynamic and morphologic assessment of the efficacy of dilation of the obstructed venous pathway.

Cerebral blood flow velocities monitored by transcranial Doppler during cardiac catheterizations in children.

Transcranial Doppler (TCD) was used to evaluate brain circulation during cardiac catheterizations in 32 children requiring pulmonary (n=10) or aortic balloon dilatations (n=2), ductus arteriosus coil insertions (n=5), or angiography (n=15). Cerebral blood flow velocity (CBFV) in the middle cerebral artery was measured before (baseline), during, and after each procedure (mean+/-95%ci). High-intensity transient signals (HITS) were also detected during these maneuvers. Balloon angioplasty decreased CBFV by 63+/-11% from baseline (P < 0.01). Shorter durations of the inflation cycle resulted in earlier CBFV recovery (r=0.78). During angiography, CBFV increased by 11+/-4% (P < 0.01) in all except one case that showed retrograde diastolic flow. Mean total HITS count was 44 (95%ci.limits: 27,74). These signals were more frequently found in septal defects or systemic arterial manipulations. Pediatric cardiac catheterization may impose transient fluctuations in brain perfusion as indicated by TCD, but their clinical implications are uncertain. CBFV changes during balloon angioplasty emphasize the importance of rapid inflation/deflation cycles. TCD can monitor such changes and evaluate preventive measures.

Systolic function of the univentricular heart: comparison of the Simpson's rule with acoustic quantification.

BACKGROUND: Acoustic quantification (AQ) is a new method of obtaining real-time information about systolic ventricular function. This method establishes a ;blood-tissue interface' and computes an intraventricular blood volume in real time to derive a beat to beat instantaneous ejection fraction. AQ assessment of systolic function has been reported previously in patients with normal cardiotypes and varying degrees of myocardial dysfunction. OBJECTIVE: To determine the potential utility of AQ in patients with abnormal ventricular morphology, in whom systolic function may be difficult to measure by traditional methods. PATIENTS AND DESIGN: Seventeen children (nine females) ranging in age from five days to 18 years (mean 6.9 years) with univentricular left ventricle heart morphology underwent a prospective and comparative echocardiographic study of ventricular function with the use of AQ and manual planimetry (single plane Simpson's rule). Imaging was done during steady state without sedation. Routine scan planes were performed, followed by repeat scanning of the univentricle from the apical four-chamber view in the AQ mode. Subsequently, manual planimetry using Simpson's rule was performed from an online graphical analysis package to measure systolic and diastolic frames from the conventional replay images. These data were used to calculate ejection fraction using standards previously established. The results were then compared with real-time AQ results. SETTING: Tertiary care referral center. RESULTS: Scan time for the combined standard and AQ imaging averaged 45 mins (range 35 to 65 mins). Measured ejection fraction by AQ and manual planimetry were 44 +/- 11% and 46 +/- 10%, respectively. Statistical analysis by repeated measures ANOVA with Bonferroni/Dunn correction (F = 0.6, df = 1,32, P = 0.44) demonstrated significant agreement between AQ and manual planimetry with an intraclass correlation coefficient of 0.93. Bland-Altman analysis was used to provide a graphic display of the clinical significance of differences in the comparison of the two methods of measurement. CONCLUSIONS: These findings support the use of AQ for continuous online determination of indexes of systolic function for patients with univentricular left ventricle morphology. The variability in the morphology inherently present within this group of patients results in a wider variability of determined ejection fraction. Particular attention must be directed to the technical aspects of image acquisition and AQ application to ensure accuracy.

Anesthetic management of patients receiving calculus therapy with a third-generation extracorporeal lithotripsy machine.

We reviewed the anesthetic requirements for satisfactory use of a third-generation electromagnetic-source design for extracorporeal shockwave lithotripsy (SWL). Medical records were reviewed for a period of 9 months on all patients receiving anesthesia care for SWL with and without other urologic procedures. The Modulith SL20 was used on 56 ASA Class I-III patients having 87 SWL treatments. Demographic and anesthetic variables were recorded. Complications documented included dysrhythmias, nausea necessitating treatment, and conversion from sedation to regional or general anesthesia. The majority of procedures (83%) were performed on an outpatient basis. Patients were classified as ASA physical status I (27%), II (63%), or III (10%). Monitored anesthesia care with intravenous sedation was utilized in 93% of cases. Of these cases, 78 involved a combination of intravenous propofol, fentanyl, and midazolam; the remaining 3 involved propofol, alfentanil, and/or midazolam. The mean treatment duration was 36 minutes. Patients were discharged within 1 hour after procedure completion in 77 cases (89%). Nausea necessitating treatment was rare (3%). The mean dose of propofol administered with SWL as the only procedure was 272 +/- 112 mg. When SWL was combined with other urologic procedures, the mean dose of propofol was 334 +/- 121 mg. Continuous intravenous propofol infusion provides excellent procedural conditions for SWL on the Modulith SL120, a third-generation lithotripter.

An approach to the neonate with sudden dysrhythmia: diagnosis, mechanisms, and management.

The clinical course of a newborn can be rendered more complex by the sudden development of a disturbance in heart rhythm and conduction. The caregiver's ability to recognize a particular dysrhythmia and its most probable mechanism is crucial for optimal management. During the past 15 years, there has been significant progress in the understanding of the electrophysiologic basis of rhythm disturbances in the newborn period. This understanding has led to more precise diagnosis and effective treatment regimens for infants with dysrhythmias. Using recent case studies from one intensive care nursery as illustrations, this article discusses the clinical presentation and electrophysiologic mechanisms of three common forms of rhythm and conduction disturbance. The discussion pertains to disturbances of rhythm in association with tachycardia only. Emphasis is placed on the electrocardiographic diagnosis and clinical management of these infants.

Responses of systemic and pulmonary veins to the presence of an intravascular stent in a swine model.

The outcome of stent implantation for children with pulmonary venous obstruction has been characterized by late reocclusion associated with a marked vessel neointimal proliferation. The purpose of this study was to compare the responses of the systemic vein and pulmonary vein to the presence of an intravascular stent, using a Yorkshire swine (N = 10) model. Under cardiopulmonary bypass, a single Palmaz stent was placed in the inferior vena cava (IVC) and right lower pulmonary vein (PV) with sacrifice at 4.9-6.1 months. Angiography and hemodynamic data were determined at 1 and 3 months post-stent implant and prior to euthanasia. All stents were found to be patent, with no difference in degree of thrombosis or neointimal formation. No statistical difference was found in the initial and final stent diameter for both inferior vena cava and pulmonary vein stents (PV initial 6.8 +/- 0.9; final 7.1 +/- 0.6) (IVC initial 10.4 +/- 1.2; final 10.4 +/- 1.2). Electron microscopy demonstrated smooth endothelialization of both pulmonary and systemic venous stent devices. No thrombosis was found on gross morphology. The data indicate that there is no intrinsic difference in the response of the pulmonary vein to the presence of a stent device. The clinical experience of restenosis following stent implantation for pulmonary vein stenosis appears to be more related to variables of final stent diameter combined with the marked intrinsic abnormal vessel architecture, as seen with this condition.

Cardiopulmonary bypass with circulatory arrest in swine: echocardiographic evaluation of left ventricle function and pulmonary vein flow.

Complete two-dimensional echocardiography and Doppler flow studies were performed on 17 swine as part of an experimental protocol involving cardiopulmonary bypass with circulatory arrest. Indices of left ventricular function and pulmonary vein morphology and flow velocity were taken before (17/17) and after bypass (11/17). A total of 70 measurements were available for analysis. Doppler flow velocity within the right pulmonary vein was 0.5 +/- 0.06 m/sec and was 0.52 +/- 0.05 m/sec in the left pulmonary vein. The swine were found to have a marked reduction in left ventricle function compared with that in previous reports, though no statistical difference was seen between pre- and post-cardiopulmonary bypass studies (preoperative shortening fraction was 21 +/- 5% and ejection fraction was 41 +/- 8%; postoperative shortening fraction was 23 +/- 5% and ejection fraction was 42 +/- 7%). Swine with shortening and ejection fractions of < 20% were found to have more unstable hemodynamics during cardiopulmonary bypass.