W. Reid Thompson

Monitoring Cerebral Blood Flow Pressure Autoregulation in Pediatric Patients During Cardiac Surgery

Background and Purpose— The limits of cerebral blood flow-pressure autoregulation have not been adequately defined for pediatric patients. Mean arterial blood pressure below these limits might contribute to brain injury during cardiac surgery. The purpose of this pilot study was to assess a novel method of determining the lower limits of pressure autoregulation in pediatric patients supported with cardiopulmonary bypass. Methods— A prospective, observational pilot study was conducted in children (n=54) undergoing cardiac surgery with cardiopulmonary bypass for correction of congenital heart defects. Cerebral oximetry index (COx) was calculated as a moving, linear correlation coefficient between slow waves of arterial blood pressure and cerebral oximetry measured with near-infrared spectroscopy. An autoregulation curve was constructed for each patient with averaged COx values sorted by arterial blood pressure. Results— Hypotension was associated with increased values of COx (P<0.0001). For 77% of patients, an individual estimate of lower limits of pressure autoregulation could be determined using a threshold COx value of 0.4. The mean lower limits of pressure autoregulation for the cohort using this method was 42±7 mm Hg. Conclusions— This pilot study of COx monitoring in pediatric patients demonstrates an association between hypotension during cardiopulmonary bypass and impairment of autoregulation. The COx may be useful to identify arterial blood pressure-dependent limits of cerebral autoregulation during cardiopulmonary bypass. Larger trials with neurological outcomes are indicated.

Pathophysiology and Therapy of Cardiac Dysfunction in Duchenne Muscular Dystrophy

Cardiac dysfunction is a frequent manifestation of Duchenne muscular dystrophy (DMD) and a common cause of death for individuals with this condition. Early diastolic dysfunction and focal fibrosis proceed to dilated cardiomyopathy (DCM), complicated by heart failure and arrhythmia in most patients. Improvements in the management of respiratory insufficiency in DMD have improved lifespan and overall prognosis, but heart failure and sudden death continue to impact survival and quality of life for people with DMD. Since the specific mechanisms resulting in heart failure for people with DMD are poorly understood, current treatments are not targeted, but rely on approaches that are considered standard for DCM. These approaches include angiotensin-converting enzyme (ACE) inhibitors and β-adrenoceptor antagonists. Data from one trial in DMD support the use of ACE inhibitors before the onset of left ventricular dysfunction. Angiotensin receptor blockers have shown similar efficacy to ACE inhibitors in numerous studies of dilated cardiomyopathy, and are a good choice for patients who cannot tolerate ACE inhibition. The pathogenesis of DMD-associated cardiomyopathy may be similar to other genetic disorders of the cytoskeletal complex of ventricular myocytes, though unique features offer targeted opportunities to impact treatment. Novel areas of investigation are focused on the regulatory role of dystrophin in relation to neuronal nitric oxide synthase (nNOS) and transient receptor potential canonical channels (TRPC). Inhibition of phosphodiesterase-5 (PDE5) addresses several aspects of regulatory dysfunction induced by dystrophin deficiency, and studies with PDE5-inhibitors have shown benefits in murine models of DMD. PDE5-inhibitors are currently under investigation in at least one study in humans. This article focuses on mechanisms of cardiac dysfunction, as well as potential targets for pharmacologic manipulation to prevent or improve cardiomyopathy in DMD.

Sildenafil does not improve cardiomyopathy in Duchenne/Becker muscular dystrophy

Duchenne and Becker muscular dystrophies (DBMD) are allelic disorders caused by mutations in dystrophin. Adults with DBMD develop life‐threatening cardiomyopathy. Inhibition of phosphodiesterase 5 (PDE5) improves cardiac function in mouse models of DBMD. To determine whether the PDE5‐inhibitor sildenafil benefits human dystrophinopathy, we conducted a randomized, double‐blind, placebo‐controlled trial (ClinicalTrials.gov, number NCT01168908).

The Impact of Computer-assisted Auscultation on Physician Referrals of Asymptomatic Patients with Heart Murmurs

As many as 50–70% of asymptomatic children referred for specialist evaluation or echocardiography because of a murmur have no heart disease.

Evaluation of Cardiac Auscultation Skills in Pediatric Residents

Auscultation skills are in decline, but few studies have shown which specific aspects are most difficult for trainees. We evaluated individual aspects of cardiac auscultation among pediatric residents using recorded heart sounds to determine which elements pose the most difficulty. Methods. Auscultation proficiency was assessed among 34 trainees following a pediatric cardiology rotation using an open-set format evaluation module, similar to the actual clinical auscultation description process. Results. Diagnostic accuracy for distinguishing normal from abnormal cases was 73%. Findings most commonly correctly identified included pathological systolic and diastolic murmurs and widely split second heart sounds. Those least likely to be identified included continuous murmurs and clicks. Accuracy was low for identifying specific diagnoses. Conclusions. Given time constraints for clinical skills teaching, this suggests that focusing on distinguishing normal from abnormal heart sounds and murmurs instead of making specific diagnoses may be a more realistic goal for pediatric resident auscultation training.

Prevalence and distribution of regional scar in dysfunctional myocardial segments in Duchenne muscular dystrophy

BackgroundThe segmental relationship between cardiovascular magnetic resonance (CMR) peak circumferential strain (Ecc) and myocardial scar has not been well characterized in Duchenne muscular dystrophy (DMD), and it is unknown whether echocardiography accurately measures Ecc in DMD. We assessed segmental Ecc and scar using CMR with myocardial tissue tagging and late gadolinium enhancement (LGE) in patients with DMD, then compared CMR with echocardiographic velocity vector imaging (VVI) for regional Ecc based on independent observer assessments.ResultsParticipants enrolled (n = 16; age 8-23) had median left ventricular (LV) ejection fraction of 0.52 (range 0.28-0.69), and 156 basal and mid-cavity myocardial segments from the 13 patients completing the LGE protocol were analyzed for strain and scar. Segmental CMR Ecc in the most negative quartile (quartile 4) ruled out scar in that segment, but scar was present in 46% of segments in the least negative (most dysfunctional) Ecc quartile 1, 33% of Ecc quartile 2 segments, and 15% of Ecc quartile 3 segments. Overall scar prevalence in inferior, inferolateral, and anterolateral segments was eight times higher than in inferoseptal, anteroseptal, and anterior segments (p < 0.001). This increased proportion of scar in lateral versus septal segments was consistent across CMR Ecc quartiles (quartile 1: 76% versus 11%, p = 0.001; quartile 2: 65% versus 9%, p < 0.001; quartile 3: 38% versus 0%, p < 0.001). Echocardiographic analysis could be performed in 12 of 14 patients with CMR exams and had to be limited to mid-cavity slices. Echo segmental Ecc in the most negative quartile made scar by CMR in that segment highly unlikely, but the correlation in segmental Ecc between CMR and echo was limited (r = 0.27; p = 0.02).ConclusionsThe relationship between scar and Ecc in DMD is complex. Among myocardial segments with depressed Ecc, scar prevalence was much higher in inferior, inferolateral, and anterolateral segments, indicating a regionally dependent association between abnormal Ecc and scar, with free wall segments commonly developing dysfunction with scar and septal segments developing dysfunction without scar. Although normal echocardiographic Ecc predicted absence of scar, regional echocardiographic Ecc by VVI has only a limited association with CMR Ecc in DMD.

Antibody-Mediated Red Blood Cell Agglutination Resulting in Spontaneous Echocardiographic Contrast

Abstract. Spontaneous echocardiographic contrast is well reported in states of low flow and low shear stress, and the primary blood component involved has been reported as red blood cells via rouleaux formation. This report describes the occurrence of spontaneous echocardiographic contrast from a unique mechanism of IgM-mediated red blood cell agglutination and describes the clinical sequelae.

What makes medical students better listeners

Diagnosing heart conditions by auscultation is an important clinical skill commonly learnt by medical students. Clinical proficiency for this skill is in decline [1], and new teaching methods are needed. Successful discrimination of heartbeat sounds is believed to benefit mainly from acoustical training [2]. From recent studies of auditory training [3,4] we hypothesized that semantic representations outside the auditory cortex contribute to diagnostic accuracy in cardiac auscultation. To test this hypothesis, we analysed auditory evoked potentials (AEPs) which were recorded from medical students while they diagnosed quadruplets of heartbeat cycles. The comparison of trials with correct (Hits) versus incorrect diagnosis (Misses) revealed a significant difference in brain activity at 280-310 ms after the onset of the second cycle within the left middle frontal gyrus (MFG) and the right prefrontal cortex. This timing and locus suggest that semantic rather than acoustic representations contribute critically to auscultation skills. Thus, teaching auscultation should emphasize the link between the heartbeat sound and its meaning. Beyond cardiac auscultation, this issue is of interest for all fields where subtle but complex perceptual differences identify items in a well-known semantic context.

Total anomalous pulmonary venous connection in a neonate characterised by low-dose, high-pitch cardiac CT.

We describe the use of low-dose dual-source cardiac for the evaluation of a neonate with suspected total anomalous pulmonary venous connection. This novel technique obviates the need for sedation or breathholding. Radiation dose-reduction strategies result in sub-mSv-estimated effective doses, substantially lower than annual background radiation dose. Low-dose, high-pitch cardiac CT should be considered as an alternative to MRI or diagnostic cardiac catheterisation in neonates with complex CHD, requiring definitive anatomic evaluation.

Cardiac History and Physical Examination

In most instances, history and physical examination provide crucial information when determining if a child has heart disease Heart disease should be suspected if history reveals: Shortness of breath without wheezing History of central cyanosis Easy fatigability Failure to thrive Family history of heart disease or sudden cardiac death Heart disease should be suspected if physical examination reveals: Central cyanosis, clubbing of digits Poor capillary refill and pulses Delayed and weak femoral pulse when compared to brachial pulse Hyperactive precordium, thrill Murmurs louder than 2/6, diastolic murmurs Single S2, fixed splitting of S2, additional heart sounds