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Biomedical subjects

J P Saul

Publications and source records attributed to J P Saul.

At least 19 recordsLinked to original sources

Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit fontan procedures.

This study compares the effects of two techniques for the Fontan procedure-intraatrial lateral tunnel (IALT) and extracardiac conduit (ECC) -on sinus node dysfunction. Between January 1992 and December 1998, 54 patients underwent a total cavopulmonary connection Fontan procedure. Of these, 36 had follow-up 24-hour Holter recordings, and they constitute the population for this study. The 24-hour Holter recordings were performed between January 1998 and March 1999 and were evaluated for sinus node dysfunction and atrial tachycardia. Clinical follow-up (18 +/- 11 months for the IALT group vs 34 +/- 19 months for the ECC group; p = 0.002) and surface electrocardiograms were also reviewed. Among the 36 patients, 19 had an IALT and 17 had an ECC Fontan procedure. The incidence of sinus node dysfunction was 4/19 (21%) in the IALT group and 10/17 (59%) in the ECC group (p = 0.04). No patient from either group had an identified episode of atrial tachycardia. No permanent pacemaker was placed in the IALT group, whereas three were placed in the ECC group, all for sinus node dysfunction. In summary, patients with both IALT and ECC had an important incidence of sinus node dysfunction. The incidence of sinus node dysfunction was higher in the ECC group, which may have been due to longer follow-up in this group. Atrial tachycardia was not observed in either group. Although the IALT group had less sinus node dysfunction than the ECC group and appeared to require less permanent pacing, these data may be too limited to serve as the criteria for choosing between these two techniques for performing the Fontan procedure.

Bradycardia↗

Idiopathic monomorphic ventricular tachycardia originating from the left aortic sinus cusp in children: endocardial mapping and radiofrequency catheter ablation.

BACKGROUND: Idiopathic repetitive monomorphic ventricular tachycardia with an inferior axis and left bundle branch block pattern typically originates from the superior right ventricular outflow tract. When indicated, radiofrequency catheter ablation is usually safe and effective. However, a left ventricular origin has been described recently in adult patients in whom ablation attempts in the right ventricular outflow tract were unsuccessful. Experience in pediatric patients is limited. PATIENTS AND METHODS: Since 1998, 13 young patients suffering from symptomatic ventricular tachycardia episodes with an inferior axis and left bundle branch block pattern underwent an electrophysiological study and radiofrequency catheter ablation. In 2 patients, age 13 and 15 years, no endocardial local electrograms preceding the surface ECG QRS complex could be recorded within the right ventricular outflow tract during ventricular ectopy. Detailed mapping within the left ventricular outflow tract and in the aortic root revealed local electrograms 25 and 53 ms earlier than the QRS complex and a 11/12 and 12/12 lead match during pacing inferior and anterior to the ostium of the left main coronary artery in the left aortic sinus cusp. Earliest activation was recorded 10 and 12 mm away from the coronary artery ostium identified angiographically. In each of the patients, one single radiofrequency current application (60 degrees C, 30 W, duration 30 and 60 s, respectively) resulted in complete cessation of ventricular ectopy. Subsequent selective injection into the left coronary artery did not reveal any abnormalities. During follow-up (2 and 34 months) off any antiarrhythmic drugs, both of the patients are in continuous normal sinus rhythm. CONCLUSION: In young patients with symptomatic idiopathic ventricular tachycardia originating from the left aortic sinus cusp, radiofrequency catheter ablation was safe and effective.

Adolescent↗

Ventricular tachycardia in nonpostoperative pediatric patients: role of radiofrequency catheter ablation.

Experience concerning radiofrequency catheter ablation (RFCA) of ventricular tachycardia (VT) in pediatric patients is limited. In adults, success rates vary widely based on the etiology of the VT. Highest success rates have been achieved in patients without structural heart disease. Between March 1998 and December 1999, five young patients (age, 5 months to 15 years; body weight, 5.5-61.6 kg) underwent RFCA for VT at our institution [structurally normal heart (n = 4), preoperative tetralogy of Fallot (n = 1)]. Monomorphic VT was present in four children, and an infant with MIDAS syndrome had polymorphic VT. Clinical presentation varied: palpitations, n = 2, congestive heart failure, n = 3. All patients had been proven to be unresponsive to one to six (median, three) antiarrhythmic drugs. In all five patients, VT could be successfully eliminated by RFCA after a total of nine (range, 1-4) procedures. Activation mapping and pace mapping were used to identify the anatomical substrate, which was located in the right ventricle/right ventricular outflow tract in all four patients with monomorphic VT and in the left ventricular septum/left ventricular free wall in the infant with polymorphic VT. There were no significant complications in any patient. During follow-up (20-42 months), all patients are in normal sinus rhythm. Left ventricular function recovered in all three patients who had initially presented with congestive heart failure. RFCA can be effective, safe, and life saving in children with medically resistant VT who have not been operated on for congenital heart disease, even when the VT is polymorphic. Although the number of patients is small, RFCA may be the treatment of choice for symptomatic VT in pediatric patients.

Adolescent↗

Radiofrequency catheter ablation in infants </=18 months old: when is it done and how do they fare?: short-term data from the pediatric ablation registry.

BACKGROUND: The objective of this study was to determine the indications, the safety, and the efficacy of pediatric radiofrequency catheter ablation (RFCA) in infants. METHODS AND RESULTS: Data from the pediatric RFCA registry were reviewed. Between August 1989 and January 1999, 137 infants, defined by age 0 to 1.5 years (median 0.7 years; weight 1.9 to 14.8 kg, median 10 kg), underwent 152 procedures in 27 of 49 registry centers (55%), compared with 5960 noninfants undergoing 6610 procedures during a comparable period. Structural heart disease was present in 36% of infants, compared with 11.2% of noninfants (P<0.0001). RFCA in infants was performed more commonly for drug resistance or life-threatening arrhythmias than in noninfants. No differences were found between infants and noninfants in success for all tachycardia substrates (87.6% versus 90.6%, P=0.11), for single accessory pathways (94.5% versus 91.5%, P=0.4), or for total (7.8% versus 7.4%, P=1) and major (4.6% versus 2.9%, P=0.17) complications. Neither success for infants with a single accessory pathway nor complications for the entire infant group were related to weight, age, center size, or the presence of structural heart disease. Centers that performed infant procedures, however, enrolled more patients overall in the registry than those that did not perform infant procedures, and successful procedures in infants were performed by more experienced physicians than failed procedures. CONCLUSIONS: Compared with noninfants, RFCA in infants is usually performed for drug resistance or life-threatening arrhythmias, often in the presence of structural heart disease. The data support the use of RFCA by experienced physicians in selected infants.

Adolescent↗

Estimation of frequency shift in cardiovascular variability signals.

Spectral analysis of heart rate (HR) and blood pressure (BP) oscillations has traditionally concentrated on spectral power, although a shift in spectral frequency characterises the variability better than power in some cases. Experimental data were obtained from 14 healthy males in control and pharmacological blockade conditions. When parasympathetic control was reduced, LF oscillations of HR and BP tend to shift towards lower frequencies. Three parameters were compared to estimate the spectral shift within the low frequency (LF, 0.04-0.15 Hz) band in HR and BP variability: mean (fmean), median (fmed), and central frequency (fc). Parameter variance (pSTD) and sensitivity to noise were also estimated using realistic HR, systolic BP (SBP) and diastolic BP (DBP) data. fmean showed the lowest parameter variance both for an autoregressive (AR) method (SBP pSTD 3.1 vs 4.8 vs 4.7 mHz for fmean, fmed and fc, respectively; p<0.001) and an FFT method (SBP pSTD 4.7 vs 7.7 mHz for fmean and fmed, respectively; p<0.001). Furthermore, fmean was least sensitive to noise. fc showed the poorest performance being especially sensitive to noise. To analyse the spectral shift, fmean is preferred, since it performs better than fc, which has been used in most previous studies. To quantify the frequency of oscillations in cardiovascular signals, the mean frequency is recommended, with analyses across different spectral bands.

Adrenergic beta-Antagonists↗

Spontaneously terminating apparent ventricular fibrillation during transesophageal electrophysiological testing in infants with Wolff-Parkinson-White syndrome.

This article describes two infants with Wolff-Parkinson-White (WPW) syndrome in whom apparent VF occurred without antecedent AF or atrial flutter during routine transesophageal electrophysiological testing. Remarkably, this arrhythmia terminated spontaneously in both infants. The documentation of self-limited apparent VF, or polymorphic ventricular tachycardia close to VF, in transesophageal testing adds another dimension to the management of WPW.

Electrocardiography↗

Atrioventricular reciprocating tachycardia involving twin atrioventricular nodes in patients with complex congenital heart disease.

INTRODUCTION: Histologic studies of autopsy specimens described the coexistence of two distinct AV nodes (so-called "Minckeberg sling" or "twin AV nodes") in specific congenital heart defects; however, the clinical electrophysiologic (EP) characteristics of twin AV nodes have not been characterized in detail. METHODS AND RESULTS: Since April 1993, a total of seven patients with complex congenital heart disease presented with AV reciprocating tachycardia suspected to be mediated by twin AV nodes. A common anatomic finding was AV discordance ([S,L,L] or [I,D,D]) with a malaligned complete AV canal defect in 5 of 7 patients. Intracardiac EP study was performed in five cases, and ablation was attempted in three patients with successful elimination of tachycardia inducibility by interruption or modification of 1 of the 2 AV nodes. Important EP characteristics included (1) the existence of two discrete nonpreexcited QRS morphologies, each with an associated His-bundle electrogram; (2) decremental as well as adenosine-sensitive anterograde and retrograde conduction; and (3) inducible AV reciprocating tachycardia with anterograde conduction over one AV nodal pathway and retrograde conduction over the alternate AV nodal pathway. The existence of two AV nodes was further supported in the group treated with radiofrequency ablation by the development of transient accelerated junctional rhythm during energy delivery with an identical QRS morphology to that generated by anterograde conduction over the targeted AV node. CONCLUSION: Reciprocating tachycardia mediated by twin AV nodes can be a source of recurrent supraventricular tachycardia in patients with specific forms of complex congenital heart disease. Successful treatment with catheter ablation is possible.

Atrioventricular Node↗

Pharmacokinetics and pharmacodynamics of sotalol in a pediatric population with supraventricular and ventricular tachyarrhythmia.

OBJECTIVE: This pharmacokinetic-pharmacodynamic study was designed to define the steady-state relationship between pharmacologic response and dose or concentration of sotalol in children with cardiac arrhythmias, with an emphasis on neonates and infants. METHODS: The treatment consisted of an upward titration with unit doses of 10, 30, and 70 mg of sotalol per square meter of body surface area. The patients received 3 doses at each dose level. The dosing interval was 8 hours. The Class III and beta-blocking activities of sotalol were derived from the QT and R-R intervals, respectively, of the surface electrocardiogram, which was recorded at 6 scheduled times before and after the third, sixth, and ninth doses. During these three dose intervals, 4 scheduled blood samples were also collected. Drug concentrations were measured with a validated nonstereoselective liquid chromatographic tandem mass spectrometric detection assay. Pharmacokinetic and pharmacodynamic parameters were obtained with standard methods. RESULTS: Twenty-one centers enrolled 25 patients in the study: 7 were neonates, 9 were infants, and 11 were children between the ages of 2 years and 12 years. The area under the drug concentration-time curve increased proportionately with dose. The apparent oral clearance of sotalol was linearly correlated with body surface area and creatinine clearance. The smallest children (body surface area <0.33 m2) displayed greater drug exposure than the larger children. The increase of QTc and R-R intervals was dose dependent. At the 70-mg/m(2) dose level, the mean (+/- standard deviation) maximum increase for the QTc interval was 14% +/- 7% and the average Class III effect during a dose interval was 7% +/- 5%. At the same dose level, the mean maximum increase of the R-R interval was 25% +/- 15% and the average beta-blocking effect during a dose interval was 12% +/- 13%. The effects tended to be larger in the smallest children. The Class III response and the plasma concentrations of sotalol were linearly related. The treatment was well tolerated. CONCLUSIONS: The steady-state pharmacokinetics of sotalol were dose proportionate. Pharmacologically important beta-blocking effects were observed at the 30-mg/m2 and 70-mg/m2 dose levels. Important Class III effects were seen at the 70-mg/m2 dose level. The Class III effect was linearly related to the drug concentration.

Anti-Arrhythmia Agents↗

Single-dose pharmacokinetics of sotalol in a pediatric population with supraventricular and/or ventricular tachyarrhythmia.

The pharmacokinetics (PK) of the antiarrhythmic sotalol, which elicits Class III and beta-blocking activity, has not been adequately defined in a pediatric population with tachyarrhythmias. The goal of this single-dose study with administration of sotalol HCl at a dose level of 30 mg/m2 body surface area (BSA) was to define the PK of the drug in the following four age groups: neonates (0-30 days), infants (1 month to 2 years), younger children (> 2 to < 7 years), and older children (7-12 years) with tachyarrhythmias of either supraventricular or ventricular origin. The drug was administered in an extemporaneously compounded syrup formulation prepared from the tablets containing sotalol HCl. For safety, vital signs and adverse events were recorded and the QTc interval and heart rate telemetrically monitored. Scheduled blood samples were taken over a 36-hour time interval following dose administration. The drug concentrations in plasma were measured by a sensitive and specific LC/MS/MS assay. Standard compartment model-independent methods were applied to compute the salient PK parameters of sotalol. Twenty-four clinical sites enrolled 34 patients. Thirty-three had analyzable data. Sotalol was rapidly absorbed, with mean peak concentrations occurring 2 to 3 hours after administration. The elimination of sotalol was characterized by an average half-life of between 7.4 and 9.2 hours in the four age groups. There existed statistically significant linear relationships between apparent total clearance (CL/f) or apparent volume of distribution (V lambda z/f) after oral administration and the covariates BSA, creatinine clearance (CLcr), body weight (BW), or age. The best predictors for CL/f were CLcr and BSA, whereas BW best predicted the V lambda z/f. The total area under the drug concentration-time curve in the smallest children with a BSA < 0.33 m2 was significantly greater than that in the larger children. This finding indicated that the BSA-based dose adjustment used in this study led to a larger exposure in the smallest children, whereas the exposure to the drug was similar in the larger children. The dose of 30 mg/m2 was tolerated well. No serious drug-related adverse events were reported. It can be concluded that the PK of sotalol in the pediatric patients depended only on body size, except for the neonates and smallest infants in whom the disposition of sotalol was determined by both body size and maturation of eliminatory processes.

Adrenergic beta-Antagonists↗

Location of acutely successful radiofrequency catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease.

Intraatrial reentrant tachycardia (IART) is common after surgery for congenital heart disease (CHD). Radiofrequency (RF) catheter ablation of IART targets anatomic areas critical to the maintenance of the arrhythmia circuit, areas that have not been well defined in this patient population. The purpose of this study was to determine the anatomic areas critical to IART circuits, defined by activation mapping and confirmed by an acutely successful RF ablation at the site. A total of 110 RF ablation procedures in 88 patients (median age 23.4 years, range 0.1 to 62.7) with CHD were reviewed. Patients were grouped according to surgical intervention: Mustard/Senning (n = 15), other biventricular repaired CHD (n = 24), Fontan (n = 43), and palliated CHD (n = 6). In first-time ablation procedures, > or = 1 IART circuits were acutely terminated in 80% of Mustard/Senning, 71% of repaired CHD, and 72% of Fontan (p = NS). The palliated CHD group underwent 1 of 6 successful procedures (17%), and this patient was excluded. The locations of acutely successful RF applications in Mustard/Senning patients (n = 14 sites) were at the tricuspid valve isthmus (57%) and at the lateral right atrial wall (43%). In patients with repaired CHD (n = 18 sites), successful RF sites were at the isthmus (67%) and the lateral (22%) and anterior (11%) right atria. In the Fontan group (n = 40 sites), successful RF sites included the lateral right atrial wall (53%), the anterior right atrium (25%), the isthmus area (15%), and the atrial septum (7%). Location of success was statistically different for the Fontan group (p = .002). In conclusion, the tricuspid valve isthmus is a critical area for ablation of IART during the Mustard/ Senning procedure and in patients with repaired CHD. IART circuits in Fontan patients are anatomically distinct, with the lateral right atrial wall being the more common area for successful RF applications. This information may guide RF and/or surgical ablation procedures in patients with CHD and IART.

Adolescent↗

Mortality following radiofrequency catheter ablation (from the Pediatric Radiofrequency Ablation Registry). Participating members of the Pediatric Electrophysiology Society.

Deaths have been reported following radiofrequency catheter ablation (RFCA), but the mortality rate in children has not been defined. This study sought to analyze the incidence and the factors associated with mortality related to RFCA. Ten of 4,651 cases (0.22%) reported to the Pediatric RFCA Registry resulting in death were reviewed and compared with a matched control group (n = 18). Death occurred in 5 of 4,092 children (0.12%, ages 0.1 to 13.3 years) with structurally normal hearts. Death was related to traumatic injury, myocardial perforation and hemopericardium, coronary or cerebral thromboembolism, and ventricular arrhythmia. All cases were left-sided (p = 0.019 vs right or septal) supraventricular arrhythmias with radiofrequency applications in the systemic atrium and/or ventricle, and all procedures were successful. Mortality occurred in 5 of 559 children (0.89%, p = 0.001 vs normals, ages 1.5 to 17.4 years) with structural heart disease. No new pathology except the mural radiofrequency lesions was seen at autopsy. Those with structurally normal hearts who died were smaller (32.7 vs 55.6 kg, p = 0.023) and had more radiofrequency applications (26.3 vs 8.7, p = 0.019) than those who survived. No differences were demonstrated for those with abnormal hearts. Operator experience was not different (deaths 103 +/- 106 vs controls 117 +/- 125, p = 0.41). Mortality associated with pediatric RFCA is rare, but is more frequent when there is underlying heart disease, lower patient weight, greater number of radiofrequency energy applications, and left-sided procedures. Operator experience does not appear to be a factor leading to mortality.

Adolescent↗

Biobehavioral pain responses in former extremely low birth weight infants at four months' corrected age.

OBJECTIVE: To compare biobehavioral responses to acute pain at 4 months' corrected age between former extremely low birth weight (ELBW) infants and term-born controls. METHODOLOGY: Measures of facial behavioral and cardiac autonomic reactivity in 21 former ELBW infants (mean birth weight = 763 g) were compared with term-born infants (n = 24) during baseline, lance, and recovery periods of a finger-lance blood collection. Further, painful procedures experienced during neonatal care were quantified in both groups. RESULTS: Overall, behavioral and cardiac autonomic responses to the lance were similar between groups. However, the ELBW group seemed to have a less intense parasympathetic withdrawal in the lance period and a more sustained sympathetic response during recovery than the control group. Further, in the recovery period, two behavioral patterns (early recovery and a late recovery) were apparent among the ELBW group. CONCLUSIONS: Biobehavioral pain responses were similar overall between both groups of infants. Subtle differences were observed in cardiac autonomic responses during the lance period and in behavioral recovery among ELBW infants. Whether these findings represent a long-term effect of early pain experience or a developmental lag in pain response remains unclear. The lack of an overall difference runs counter to previously reported findings of reduced behavioral response in former ELBW infants. biobehavioral pain response, premature infants, repetitive pain, heart rate variability.

Blood Specimen Collection↗

Cerebral intravascular oxygenation correlates with mean arterial pressure in critically ill premature infants.

OBJECTIVES: Premature infants experience brain injury, ie, germinal matrix-intraventricular hemorrhage (GMH-IVH) and periventricular leukomalacia (PVL), in considerable part because of disturbances in cerebral blood flow (CBF). Because such infants are susceptible to major fluctuations in mean arterial blood pressure (MAP), impaired cerebrovascular autoregulation would increase the likelihood for the changes in CBF that could result in GMH-IVH and PVL. The objectives of this study were to determine whether a state of impaired cerebrovascular autoregulation could be identified reliably and conveniently at the bedside, the frequency of any such impairment, and the relation of the impairment to the subsequent occurrence of severe GMH-IVH and PVL. PATIENTS AND METHODS: To monitor the cerebral circulation continuously and noninvasively, we used near-infrared spectroscopy (NIRS) to determine quantitative changes in cerebral concentrations of oxygenated hemoglobin (HbO(2)) and deoxygenated hemoglobin (Hb) from the first hours of life. Our previous experimental study showed a strong correlation between a measure of cerebral intravascular oxygenation (HbD), ie, HbD = HbO(2) - Hb, determined by NIRS, and volemic CBF, determined by radioactive microspheres. We studied 32 very low birth weight premature infants (gestational age: 23-31 weeks; birth weight: 605-1870 g) requiring mechanical ventilation, supplemental oxygen, and invasive blood pressure monitoring by NIRS from 1 to 3 days of age. MAP measured by arterial catheter pressure transducer and arterial oxygen saturation measured by pulse oximetry were recorded simultaneously. The relationship of MAP to HbD was quantitated by coherence analysis. RESULTS: Concordant changes (coherence scores >. 5) in HbD and MAP, consistent with impaired cerebrovascular autoregulation, were observed in 17 of the 32 infants (53%). Eight of the 17 infants (47%) developed severe GMH-IVH or PVL or both. Of the 15 infants with apparently intact autoregulation, ie, coherence scores <.5, only 2 (13%) developed severe ultrasonographic lesions. Thus, for the entire study population of 32 infants, 8 of the 10 with severe lesions exhibited coherence scores >.5. CONCLUSIONS: We conclude that NIRS can be used in a noninvasive manner at the bedside to identify premature infants with impaired cerebrovascular autoregulation, that this impairment is relatively common in such infants, and that the presence of this impairment is associated with a high likelihood of occurrence of severe GMH-IVH/PVL.

Blood Pressure↗

Catheter ablation of accessory pathways, atrioventricular nodal reentrant tachycardia, and the atrioventricular junction: final results of a prospective, multicenter clinical trial. The Atakr Multicenter Investigators Group.

BACKGROUND: The purpose of this study was to evaluate the safety and efficacy of a temperature-controlled radiofrequency catheter ablation system. METHODS AND RESULTS: The patient population included 1050 patients who had undergone ablation of atrioventricular nodal reentrant tachycardia (AVNRT), an accessory pathway (AP), or the atrioventricular junction (AVJ). Ablation was successful in 996 patients. The probability of success was highest among patients who had undergone ablation of the AVJ, lowest in patients who had undergone ablation of an AP, and in between for patients who had undergone ablation of AVNRT. A major complication occurred in 32 patients. Four variables predicted ablation success (AVJ, AVNRT, or left free wall AP ablation and an experienced center). Four factors predicted arrhythmia recurrence (right free wall, posteroseptal, septal, and multiple APs). Two variables predicted development of a complication (structural heart disease and the presence of multiple targets), and 3 variables predicted an increased risk of death (heart disease, lower ejection fraction, and AVJ ablation). CONCLUSIONS: These findings may serve as a guide to clinicians considering therapeutic options in patients who are candidates for ablation.

Adolescent↗

Value of programmed ventricular stimulation in patients with congenital heart disease.

INTRODUCTION: The role of programmed ventricular stimulation (VSTIM) for risk stratification in congenital heart disease is unclear. We analyzed the results of VSTIM in selected congenital heart disease survivors at a single center to determine whether it improved the ability to predict a serious outcome. METHODS AND RESULTS: Between July 1985 and September 1996, 140 primary VSTIM studies were performed on 130 patients (median age 18.1 years, range 0 to 51). Tetralogy of Fallot (33 %), d-transposition of the great arteries (25 %), and left ventricular outflow tract obstruction (12%) accounted for the majority of patients. Indications included spontaneous ventricular tachycardia (VT) of > or = 3 beats (72%) and/or symptoms (68%). Sustained VT was induced in 25% of the studies, and nonsustained VT in 12%. Atrial flutter or other supraventricular tachycardia was documented in 32% and bradyarrhythmias in 26%. By univariate analysis, mortality was increased in patients with positive VSTIM versus negative VSTIM (18% vs 7%, P = 0.04). Using multivariate analysis, positive VSTIM was associated with a sixfold increased risk of decreased survival and a threefold increased risk of serious arrhythmic events, allowing up to 87% sensitivity in predicting mortality. However, 7 (33%) of 21 patients with documented clinical VT had false-negative studies. CONCLUSION: VSTIM in a large, selected group of congenital heart disease patients identified a subgroup with significantly increased mortality and sudden arrhythmic events. Failure to induce VT was a favorable prognostic sign, but the frequency of false-negative studies was high. Frequent supraventricular tachycardia further complicated risk stratification. Although VSTIM appears to be a reasonable tool for evaluation of this population, a larger, multicenter trial is recommended to clarify its utility.

Adolescent↗

Preventing sudden death after repair of tetralogy of Fallot: complex therapy for complex patients.

Sudden arrhythmic death in patients with repaired tetralogy of Fallot or its variants has a variety of causes. Consequently, it can serve as a paradigm for management of potentially malignant arrhythmias in all pediatric patients, particularly with regard to the use of nonpharmacologic therapy for management. Five cases are presented as touchpoints for discussion and demonstrate a number of important issues concerning the assessment and reduction of sudden cardiac death risk in these patients. First, there are no clinical parameters that can be used to accurately assess risk. Second, pharmacologic agents alone rarely are adequate therapy. Third, catheter ablation and antitachycardia devices continue to play an ever increasing role in management of these patients, and, finally, additional data are necessary to establish clear management guidelines in patients with congenital heart disease at risk for arrhythmic death.

Adolescent↗

The developmental character of cardiac autonomic responses to an acute noxious event in 4- and 8-month-old healthy infants.

Heart rate (HR) has been widely studied as a measure of an individual's response to painful stimuli. It remains unclear whether changes in mean HR or the variability of HR are specifically related to the noxious stimulus (i.e. pain). Neither is it well understood how such changes reflect underlying neurologic control mechanisms that produce these responses, or how these mechanisms change during the first year of life. To study the changes in cardiac autonomic modulation that occur with acute pain and with age during early infancy, the relationship between respiratory activity and short-term variations of HR (i.e. respiratory sinus arrhythmia) was quantified in a longitudinal study of term born healthy infants who underwent a finger lance blood collection at 4 months of age (n = 24) and again at 8 months of age (n = 20). Quantitative respiratory activity and HR were obtained during baseline, lance, and recovery periods. Time and frequency domain analyses from 2.2-min epochs of data yielded mean values, spectral measures of low (0.04-0.15 Hz) and high (0.15-0.80 Hz) frequency power (LF and HF), and the LF/HF ratio. To determine sympathetic and parasympathetic cardiac activity, the transfer relation between respiration and HR was used. At both 4 and 8 months, mean HR increased significantly with the noxious event (p > 0.01). There were age-related differences in the pattern of LF, HF, and LF/HF ratio changes. Although these parameters all decreased (p > 0.01) at 4 months, LF and LF/HF increased at 8 months and at 8 months HF remained stable in response to the noxious stimulus. Transfer gain changes with the lance demonstrated a change from predominant vagal baseline to a sympathetic condition at both ages. The primary finding of this study is that a response to an acute noxious stimulus appears to produce an increase in respiratory-related sympathetic HR control and a significant decrease in respiratory-related parasympathetic control at both 4 and 8 months. Furthermore, with increasing age, the sympathetic and parasympathetic changes appear to be less intense, but more sustained.

Aging↗