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Biomedical subjects

R A Jonas

Publications and source records attributed to R A Jonas.

At least 37 records · Page 2Linked to original sources

Utility and limitations of near-infrared spectroscopy during cardiopulmonary bypass in a piglet model.

Near-infrared spectroscopy assessment of cytochrome oxygenation could be a valuable technique to monitor cerebral intraneuronal oxygen delivery during cardiopulmonary bypass. However, the validity of the cytochrome signal has been questioned as it could easily be overwhelmed by the Hb signal. Five- to six-week-old control piglets (n = 5) underwent cardiopulmonary bypass at 37 degrees C. Study animals (n = 10) received 100 mg/kg of sodium cyanide to uncouple cytochrome from HB: Hematocrit was then decreased in steps of 5% from 35 to 5% with crystalloid hemodilution. In study piglets, the initiation of cardiopulmonary bypass was associated with oxygenated Hb increasing from 0 to 62.9 +/- 25.6 microM times the differential path-length factor, and oxidized cytochrome a,a3 increasing to 1.9 +/- 1.8 microM times the differential path-length factor. Cyanide caused oxygenated Hb to increase further to 132.3 +/- 48.9 microM times the differential path-length factor, and oxidized cytochrome c decreased to -7.0 +/- 2.6 microM times the differential path-length factor as anticipated, confirming uncoupling of electron transport. However, hemodilution subsequently resulted in linear decreases in oxidized cytochrome a,a3 (F = 8.57, p < 0.001) suggesting important cross-talk between the Hb and cytochrome signals as cytochrome is only intracellular. In control piglets, tissue oxygenation index showed a positive correlation with pump flow (r = 0.986, p = 0.013). The cytochrome signal as presently measured by near-infrared spectroscopy is highly dependent on hematocrit.

Animals↗

Management of transposition of the great arteries with single coronary artery.

Approximately 5% of patients with transposition of the great arteries have a single coronary artery. In the early years of the arterial switch procedure a single coronary artery was associated with an increased risk of mortality. This was particularly true for the most common type of single coronary artery in which there is a right posterior ostium giving rise to a right coronary artery that passes to the right atrioventricular groove and a left coronary artery that passes posterior to the pulmonary artery. An understanding of the mechanisms by which this coronary pattern increases the risk of mortality has led to neutralization of this risk factor. The risk of late reintervention after an arterial switch with single coronary artery is increased with a single left posterior ostium with the right coronary passing anterior to the aorta. The mechanism remains unclear.

Coronary Vessels↗

Cognitive development after the Fontan operation.

BACKGROUND: Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure. METHODS AND RESULTS: A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3. 7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7+/-17.4 (P<0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores <70 (P=0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (P=0.002), the anatomic diagnoses of hypoplastic left heart syndrome (P<0.001) and "other complex" (P=0.05), and prior placement of a pulmonary artery band (P=0.04). Mean composite achievement score was 91.6+/-15. 4 (P<0.001 versus normal); 14 patients (10.8%) scored <70 (P<0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (P=0.004) and "other complex" (P=0.003) or prior use of circulatory arrest (P=0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (P=0.01). CONCLUSIONS: Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population.

Adolescent↗

Minimal access approach for the repair of atrial septal defect: the initial 135 patients.

BACKGROUND: From May 1996 to August 1998 a minimal access approach was used for 135 of 200 consecutive surgical atrial septal defects closures in children through young adults ranging in age from 6 months to 25 years (median 5 years). METHODS: A 3.5- to 5-cm midline incision was centered over the xiphoid with division of the xiphoid alone (transxiphoid) or of the lower sternum (ministernotomy); both groups underwent bicaval venous cannulation through the incision. Cardioplegia and aortic cross-clamping were administered through the incision. Cephalad retraction of the sternum with a fixed-arm retractor aided exposure. RESULTS: There have been no early or late deaths and no bleeding or wound complications. No procedure required conversion to a full sternotomy, and no cannulation attempt was abandoned for an alternate site. Cross-clamp and cardiopulmonary bypass times were equivalent to those in the full sternotomy group. The mean length of hospital stay in the ministernotomy group was 2.7 days. CONCLUSIONS: The closure of atrial septal defects can be performed through a transxiphoid or ministernotomy approach, conferring a satisfactory cosmetic result without compromising the safety or accuracy of the repair.

Adolescent↗

Long-term follow-up of pediatric cardiac patients requiring mechanical circulatory support.

BACKGROUND: The present study examines the long-term outcome of pediatric patients with cardiac disease who required mechanical circulatory support with extracorporeal membrane oxygenation or ventricular assist devices. METHODS: Telephone interviews and questionnaires were administered to parents and physicians of pediatric cardiac patients who were in-hospital survivors after requiring mechanical circulatory support, with either extracorporeal membrane oxygenation or ventricular assist devices. Data was collected regarding these patients' general health, cardiac status, and neurologic outcome, and compared between the two modes of support. RESULTS: Follow-up was available for 26 patients supported with extracorporeal membrane oxygenation (25 survivors, 96%) and 11 patients supported with ventricular assist devices (10 survivors, 91%); median follow-up 42 months, 11 to 92 months). More than 80% of survivors were in New York Heart Association class I or II. Of 31 patients for whom neurologic assessment data was available, moderate to severe neurologic impairment was more common for extracorporeal membrane oxygenation supported patients [13 of 21, 59%) than for ventricular assist device supported patients (2 of 10, 20% p = 0.03). Neurologic impairment was associated with small patient size and the use of circulatory arrest during cardiac surgical repair, but was not associated with in-hospital neurologic complications, carotid cannulation, or presupport cardiac arrest. CONCLUSIONS: The long-term survival and cardiac functional status of pediatric cardiac patients requiring mechanical circulatory support is favorable. Extracorporeal membrane oxygenation supported patients demonstrate higher rates of neurologic impairment than patients supported with ventricular assist devices. Poor neurologic outcomes are associated with institution of support in younger patients with more complex congenital heart disease.

Adolescent↗

Utility of near-infrared spectroscopic measurements during deep hypothermic circulatory arrest.

BACKGROUND: Near-infrared spectroscopy (NIRS) is used to monitor cerebral oxygenation during cardiac surgery. However, interpretation of the signals is controversial. The aim of the study was to determine which NIRS variable best correlated with brain damage as assessed by animal behavior and neurohistologic score and to compare the accuracy of NIRS and magnetic resonance spectroscopy (MRS) in predicting brain injury. METHODS: Forty 5-week-old piglets underwent 60 minutes of deep hypothermic circulatory arrest (DHCA) at 15 degrees C. Changes in brain adenosine triphosphate (ATP), phosphocreatine (PCr), and intracellular pH (pHi) were determined by MRS and correlated to changes in oxygenated hemoglobin (HbO2), deoxygenated hemoglobin (Hb), and oxidized cytochrome a,a3 (CytOx) NIRS signals. Brains were fixed on day 4 and examined using a neurohistologic score. RESULTS: Reductions in CytOx and HbO2 values were correlated closely with decreases in ATP, PCr, and pHi. The changes in CytOx and PCr showed the strongest correlation (r = 0.623). Maximal CytOx reduction during DHCA of more than -25 microM * differential pathlength factor (DPF) predicted brain damage with a sensitivity of 100% and a specificity of 75%. The histologic score was also correlated with a decrease in ATP (r = -0.52 for CytOx; r = -0.32 for ATP); HbO2, PCr, and pHi showed no correlations. CONCLUSIONS: Reduction in CytOx correlates with decreased brain energy state and predicts histologic brain injury after DHCA with a high sensitivity. These data suggest that the level of CytOx could be a very important predictor of brain damage during DHCA.

Adenosine Triphosphate↗

Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus.

OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.

Abnormalities, Multiple↗

Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations.

OBJECTIVE: We compared the Norwood stage I operation for hypoplastic left heart syndrome and other complex malformations with ductus-dependent systemic circulation. METHODS: A retrospective study of 194 patients who underwent a Norwood stage I palliation between 1990 and 1998 was conducted. Malformations in 131 patients were classified as hypoplastic left heart syndrome, defined as aortic and mitral atresia or severe stenosis, normal segmental anatomy, intact ventricular septum, and hypoplasia of the left ventricle. Sixty three patients had other lesions: hypoplastic left ventricle with ventricular septal defect (n = 18), unbalanced complete atrioventricular canal (n = 9), complex double-outlet right ventricle (n = 14), double-inlet left ventricle (n = 11), tricuspid atresia with transposition of the great arteries (n = 6), and others (n = 5), including heterotaxia. RESULTS: Operative (>30 days) and 1-year survivals were lower for patients with hypoplastic left heart syndrome than for those with other lesions (63.4% vs 81%, P =.008, and 51.2% vs 71.4%, P =.02, respectively). The presence of a nonhypoplastic left ventricle (n = 27) was associated with higher operative and 1-year survivals (96.3% vs 64.7%, P =.002; 88.9% vs 52. 7%, P <.001). A restrictive atrial septal defect and prematurity tended to increase mortality across both groups. Cox proportional hazards regression indicated that a single right ventricle was the most important independent predictor of death (P <.001). Operative mortality for all patients undergoing the stage I procedure decreased from 38.5% (1990-1994) to 21.4% after 1994 (P =.02). CONCLUSIONS: The survival of patients with malformations other than hypoplastic left heart syndrome after the Norwood procedure is greater than for those with hypoplastic left heart syndrome. Staged palliation is valid surgical therapy in these patients, with good results in intermediate follow-up.

Abnormalities, Multiple↗

Surgical management of complex and tunnel-like subaortic stenosis.

BACKGROUND: Relief of primary or secondary subaortic stenosis (SAS) remains a surgical challenge. Heart block, aortic valve regurgitation and recurrent obstruction have been persistent problems. METHODS: Forty six patients who underwent surgery for complex and tunnel-like SAS between January 1990 and November 1998 were reviewed. In 45 of the 46 patients SAS developed following repair of a primary congenital heart defect and only one patient presented with de novo tunnel-like SAS. Fifteen of the 45 patients had undergone repair of double-outlet right ventricle (DORV) and the remaining 30 had undergone repair of a variety of defects. The median age at the time of surgery was 5 years. The modified Konno procedure was performed in 15 patients, Konno procedure in three, Ross-Konno procedure in two and resection of the conal septum in 12 patients. Five patients with DORV underwent replacement of the intraventricular baffle and two patients underwent an aortic valve-preserving procedure in conjunction with mitral valve replacement. RESULTS: There were no deaths. None of the patients had an exacerbation of aortic regurgitation and none developed complete heart block. The median follow-up was 3 years (range 1 month-8.5 years). Two patients developed recurrent SAS defined as a gradient of 40 mmHg or greater diagnosed by transthoracic echocardiography. Freedom from SAS at 1, 3 and 5 years was 100, 94 and 86%, respectively. CONCLUSIONS: We favor the modified Konno procedure and conal resection to the Konno or the Ross procedure, since insertion of a prosthetic valve or homograft is avoided and aortic valve function is preserved. Excellent relief of tunnel-like SAS can be achieved without damage to the conduction tissue.

Aortic Stenosis, Subvalvular↗

Cardiac surgery and neurological injury in children.

The freedom of children from vascular disease makes them an ideal population in which to study the deleterious effects of cardiopulmonary bypass. Cerebral embolic injury arises intraoperatively from air in the cardiac chambers and monitoring lines, particularly those in the left atrium. Postoperative emboli are more common in patients with single ventricle physiology, especially following a bidirectional Glenn shunt or Fontan procedure. Poor cannula selection or placement during bypass can cause maldistribution of the perfusate and cerebral injury. Postoperative hyperthermia can cause brain damage. Results of prospective and retrospective studies of neurological and developmental outcome after paediatric cardiac surgery at the Children's Hospital, Boston, have led to the following inferences: the use of circulatory arrest should be minimised; the pH stat strategy is preferred for cardiopulmonary bypass over the alpha stat strategy. A higher haematocrit on bypass, for example 30%, may be preferable to 20%; postoperative fever should be strenuously avoided. Surgeons should be aware that cardiopulmonary bypass parameters can have subtle but important effects on late developmental outcome.

Journal Article↗

Optimizing cerebral oxygenation during cardiac surgery: a review of experimental and clinical investigations with near infrared spectrophotometry.

OBJECTIVES: Near-infrared spectrophotometry (NIRS) is a promising method for non-invasive monitoring of cerebral oxygenation and hemodynamics. This paper reviews studies in which we aimed to validate NIRS in an experimental model of cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA) (validation study), use the method in experimental settings to optimize cerebral oxygenation during CPB (oxygenation study), and test its utility during routine cardiac surgery (clinical study). METHODS: Validation study: Forty 8-10 kg piglets underwent 60 min of DHCA at 15 degrees C, were extubated on the first postoperative day, and sacrificed on the 4th postoperative day for histologic investigations. During CPB and DHCA, the animals were investigated by NIRS (monitoring of cerebral oxygenated hemoglobin (HbO2) and oxidized cytochrome aa3 (Cytox)) and magnetic resonance spectroscopy (MRS) (monitoring of cerebral adenosine triphosphate (ATP) and phosphocreatine (PCr). Oxygenation study: A normoxic (n = 5) and a hyperoxic group (n = 5) of piglets underwent 120 min of DHCA and 6 h of reperfusion with NIRS monitoring. Neuronal damage was evaluated by histology. Clinical study: Patients (n = 41) undergoing routine cardiac surgery were investigated by NIRS and neuropsychological testings. RESULTS: Validation study: Reductions of CytOx and HbO2 values were closely correlated with decreases in ATP, PCr, and pHi. The changes in CytOx and PCr showed the strongest correlation (r = 0.623). Maximal CytOx reduction during DHCA predicted brain damage. Oxygenation study: Histology revealed a significant increase in brain damage in the normoxic group. Cytox and HbO2 tended to be lower during DHCA (p = 0.16). Clinical study: During CPB, arterial PCO2, pH and temperature were closely related to CytOx and HbO2. Patients who suffered from neuropsychological deficits had a lower CytOx minimum compared to those without these. CONCLUSIONS: NIRS measurements, especially the CytOx signal, correlate well with high energy phosphates and have a high sensitivity to predict histologic brain damage. NIRS is an important and useful tool in studies investigating cerebral oxygenation during CPB. The CytOx signal predicted impaired neuropsychological outcome in patients. Therefore, the CytOx signal holds much interest for future studies.

Animals↗

Surgery for bilateral outflow tract obstruction in elastin arteriopathy.

OBJECTIVE: A number of patients with Williams syndrome or other forms of elastin arteriopathy have stenoses of pulmonary arteries in addition to supravalvular aortic stenosis. We sought to investigate the effect of the degree of pulmonary arterial stenosis on the prognosis after an operation for supravalvular aortic stenosis to help define the optimal treatment strategy for patients with severe forms of elastin arteriopathy. METHODS: Between 1960 and 1999, 33 patients underwent operations for supravalvular aortic stenosis while having significant stenoses of the pulmonary arteries. We retrospectively reviewed patient charts, obtained current follow-up information, and determined risk factors for survival and reoperation. RESULTS: Fifteen patients with moderate right-sided obstructions (confirmed by pulmonary artery Z-scores and right ventricular/descending aortic pressure ratio) underwent operations for supravalvular aortic stenosis only. Eighteen patients had more severe right-sided obstructions and underwent surgical relief of pulmonary arterial stenoses or right ventricular outflow tract obstruction in addition to operations for supravalvular aortic stenosis. Eight patients had undergone preoperative balloon dilations of stenotic pulmonary arteries. There were 6 early deaths and 1 late death in our series. Survival at 10 and 20 years was 76% (70% confidence interval, 68%-84%) and freedom from reintervention was 59% (70% confidence interval, 46%-71%) at 10 years and 49% (70% confidence interval, 35%-62%) at 20 years. Multivariate analysis revealed that patients with a right ventricular/descending aortic pressure ratio of 1.0 or more were at higher risk for reintervention but not for death. CONCLUSIONS: Surgical treatment of pulmonary artery obstructions in elastin arteriopathy is palliative but, in conjunction with balloon dilation of peripheral pulmonary arteries, offers good long-term survival to patients with the severest form of elastin arteriopathy.

Adolescent↗

Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation.

OBJECTIVE: Low-velocity and nonlaminar flow patterns in the Fontan circulation, as well as abnormal liver function in some patients, may partly account for the coagulation abnormalities seen. We examined (1) coagulation factor abnormalities before and after the Fontan procedure and (2) regional coagulation factor abnormalities in the Fontan circulation. METHODS: Levels of factors V, VII, VIII, X, antithrombin III, prothrombin fragment F1+2, protein C, and protein S were measured in 2 groups of patients: In 14 patients undergoing the Fontan procedure, blood was analyzed before the operation and 5 days after the operation (group 1). The median age in this group was 3.2 years. In 10 patients who had undergone the Fontan procedure, cardiac catheterization was performed and samples were taken from the femoral vein, inferior vena cava, right atrium, and pulmonary artery (group 2). The median age in this group was 6.2 years and the median follow-up from the Fontan procedure was 4.1 years. RESULTS: In group 1 a significant increase was noted postoperatively in the concentration of factor VIII (P<.001), factor X (P<.001), and prothrombin fraction F1+2 (P <.001). A significant decrease in the levels of antithrombin III (P <.001), protein C (P<.004), and protein S (P<.02) was also found. The increase in factors VIII and X persisted at 4 years' follow-up in group 2 patients. In group 2, no significant regional differences were observed between the coagulation factors measured at different sites. CONCLUSIONS: There is an increased tendency toward coagulation after the Fontan procedure. A prothrombotic state is supported by thrombin generation associated with reduced antithrombin III concentration. This increase in coagulation may contribute to the early and late risks of thromboembolism observed after the Fontan procedure. We did not find any regional differences in coagulation abnormalities in patients late after the Fontan procedure. Therefore, the mechanisms and causes of the coagulation abnormalities remain unclear.

Antithrombin III↗

Developmental outcome after surgical versus interventional closure of secundum atrial septal defect in children.

BACKGROUND: The assessment of the impact of cardiopulmonary bypass (CPB) on developmental outcomes in children who undergo open heart surgery is hampered by the absence of a suitable comparison group. The development of interventional catheterization techniques for the repair of certain types of congenital heart lesions provides the opportunity to study children who have not been exposed to CPB. METHODS AND RESULTS: We performed standardized neuropsychological testing on children after closure of a secundum atrial septal defect through the use of surgery (n=26) or a transcatheter device (n=19). Device patients, compared with surgical patients, were similar in age at defect closure (mean, 6 years) but older at follow-up testing (12.3 versus 10.6 years). The mean weight percentile at closure was greater and the defect size was smaller in the device patients. Families of device patients tended to have a higher parent IQ, higher level of maternal education, and higher level of maternal occupation. In general, however, children's IQ and achievement scores were in the normal range for both groups. In regression analyses with adjustment for age at testing and parent IQ, surgical repair was associated with a 9.5-point deficit in Full-Scale IQ (P=0. 03) and a 9.7-point deficit in Performance IQ (P=0.05). Block Design was the IQ subtest on which treatment groups differed the most (P=0. 01). Surgical patients achieved significantly better scores on errors of commission (P=0.05) and attentiveness index (P=0.03) on a continuous performance test of attention. Scores on tests of achievement and other neuropsychological domains did not differ significantly between the groups. Regression analyses within the surgical group failed to identify significant CPB-related risk factors. CONCLUSIONS: A prospective randomized trial or a study that includes prerepair and postrepair assessments is necessary to establish whether the observed advantages of device closure in neuropsychological outcome represent deleterious effects of CPB or a methodological artifact.

Cardiac Catheterization↗

Repair of tetralogy of Fallot in neonates and young infants.

BACKGROUND: The timing of repair of tetralogy of Fallot (TOF) remains controversial. Advantages to early complete repair include removal of right ventricular outflow tract obstruction, alleviation of systemic hypoxia, and avoidance of palliation with an arteriopulmonary shunt. METHODS AND RESULTS: This is a retrospective review of 99 children with TOF pulmonary stenosis (TOF/PS) or TOF pulmonary atresia (TOF/PA) who were <90 days of age undergoing early complete repair. Fifty-nine were prostaglandin E dependent, and 91% of neonates were symptomatic at the time of repair. Univariate and multivariate analyses of patient characteristics, anatomic features, and operative management showed the diagnosis of TOF/PA and smaller body surface area to be the only independent risk factors for death. Early mortality was 3% (3 of 99), and actuarial survival rates were 94% at 1 year and 91.6% at 5 years. Freedom from catheterization was 86% at 1 year and 73% at 5 years. Patients repaired for TOF/PA had a significantly lower freedom from reoperation than did those repaired for TOF/PS. CONCLUSIONS: Early complete TOF repair can be accomplished with a low mortality. Children with TOF/PA repaired had a lower freedom from reoperation that did those with TOF/PS. Longer follow-up, with emphasis on arrhythmias and right ventricular function, is required to define the long-term benefits of early repair.

Cardiac Surgical Procedures↗