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Biomedical subjects

J F Keane

Publications and source records attributed to J F Keane.

At least 55 records · Page 3Linked to original sources

Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects.

BACKGROUND: From 1958 to 1969, 1,280 patients (mostly children) with ventricular septal defects (VSDs) were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization. Most with small defects and Eisenmenger's syndrome were managed medically; most with large VSDs were managed surgically. Of those with moderate-size defects, some were managed medically, and some were managed surgically. Most had a second catheterization at the conclusion of NHS-1. More than 15 years have elapsed since NHS-1, and most of the cohort are adults. This report (Second Natural History Study) addresses the long-term results of medical and surgical management. METHODS AND RESULTS: Of an original cohort of 1,280 patients, 1,099 were alive at completion of NHS-1. New data were obtained on 976 (76.3%) of the original cohort. Probability of 25-year survival was 87%, and admission severity was the best predictor of survival. Of the 860 patients managed medically during NHS-1, 245 subsequently required surgical closure of the VSD. Only 5.5% of patients who had surgical closure required a second operation. On follow-up, there was a higher-than-normal prevalence of serious arrhythmias. Bacterial endocarditis occurred rarely. Of patients with small VSDs, 94.1% were in New York Heart Association functional class I. With the exception of those with Eisenmenger's syndrome, most patients had a final clinical status that was excellent or good. CONCLUSIONS: The majority of patients fared well. However, there was a higher-than-normal prevalence of serious arrhythmia and sudden death, including those with small VSDs.

Adolescent↗

Second natural history study of congenital heart defects. Materials and methods.

Results of the location and recruitment efforts and comparisons of responses from patients who cooperated at different levels in the Second Natural History Study of Congenital Heart Defects are included because they influenced the choice of analytic methods and are essential to the generalizability of the results to the entire study cohort. Included are examination and data collection protocols (e.g., protocol definitions, test procedures, and data editing), statistical methods (e.g., box plots, survival curves, multivariable models, and rate adjustment), participation results (e.g., proportional odds analysis, mortality, location, recruitment, and full participants, including comparison of questionnaire responses and comparison of questionnaire response and physician history), and a discussion.

Data Collection↗

Second natural history study of congenital heart defects. Quality of life of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

BACKGROUND: Quality of life of patients with congenital heart defects is an important aspect of the assessment of outcome of medical and surgical treatment. METHODS AND RESULTS: All participants in the Second Natural History Study of Congenital Heart Defects who completed the study questionnaire were included in this analysis. The questionnaire completed by the patients included inquiries relative to medical history, marital and family life, self-perception of well-being, insurability, and employability. The following general observations were apparent. The study cohort reported a self-perception of health status that was similar to that of the general population. Second, 35-40% of patients had not had a cardiac evaluation within the previous 10 years. Third, 16% of the patients had no health insurance, and 35.7% of the patients had no life insurance. For the patients with pulmonary stenosis and ventricular septal defect, the percent who were married was less than that of the corresponding national age- and sex-specific population. For patients with aortic stenosis, the percent married was greater than that of the general population for some age groups. The proportion of divorced or separated individuals was similar to that of the general population. For all three defects, the level of educational attainment exceeded the national average. Last, unemployment rates were similar to national averages except for women with aortic stenosis, who had a significantly higher unemployment rate than did age- and sex-matched controls. CONCLUSIONS: Patients initially identified with aortic stenosis, pulmonary stenosis, or ventricular septal defect in 1959-1973 have a quality of life in the mid-1980s similar to that of the general US population.

Adult↗

Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring.

BACKGROUND: Arrhythmias are a significant source of morbidity and mortality in patients with congenital heart defects. METHODS AND RESULTS: As part of the Second Natural History Study of Congenital Heart Defects (NHS-2), 24-hour ambulatory ECG monitoring was performed for full participants in the study. At least 15.5 hours of monitoring was required for inclusion in the analysis. This was achieved for 755 (90.6%) of the patients. Multiform premature ventricular contractions, ventricular couplets, and ventricular tachycardia were considered "serious arrhythmias." For patients with aortic stenosis, the presence of "serious arrhythmias" was associated with higher left ventricular end-diastolic pressure, presence of aortic regurgitation, male sex, and presence of prior aortic valve replacement. For patients with pulmonary stenosis, only older age on admission to NHS-1 was significantly associated with "serious arrhythmias." For patients with ventricular septal defect, the variables associated with "serious arrhythmias" were different for medically and surgically managed patients. For medically managed patients, higher mean pulmonary artery pressure on admission to NHS-1 and older age on admission to NHS-2 were associated with the presence of serious arrhythmias. For surgically managed patients, higher New York Heart Association functional class and cardiomegaly were associated with serious arrhythmias. CONCLUSIONS: The prevalence of arrhythmias (especially ventricular arrhythmias) was higher for patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect than for an historical control population. "Serious arrhythmias" were most frequently noted in patients with aortic stenosis who also had a higher incidence of sudden death. The prevalence of "serious arrhythmias" was second highest for patients with ventricular septal defect who had the second highest incidence of sudden death.

Adult↗

Transcatheter closure of atrial and ventricular septal defects.

The clamshell double umbrella (Bard Clamshell Septal Umbrella, USCI Division, C. R. Bard, Billerica, MA) was developed to allow closure of septal defects, both atrial and ventricular. The device and delivery system and the techniques for implanting the device in atrial and ventricular defects are described in detail. Although fractures of the arms supporting the umbrellas in some patients have lead to modification of the device, the early experience in clinical trails are encouraging.

Cardiac Catheterization↗

Transcatheter umbrella closure of valvular and paravalvular leaks.

OBJECTIVES: Our aim was to adapt the technique of transcatheter umbrella closure of intracardiac defects for closure of valvular and paravalvular defects. BACKGROUND: The double-umbrella device developed by Rashkind and Cuaso has been safely and effectively delivered across a host of intracardiac defects, but transcatheter closure of valvular and paravalvular leaks has not been reported. METHODS: Between February 1987 and September 1990, eight patients who were believed to be poor operative candidates were taken to the catheterization laboratory for transcatheter double-umbrella closure of a valvular or a paravalvular leak. Four patients had a paravalvular leak around a prosthetic aortic valve. The other four patients had a valvular leak: one patient with a regurgitant native aortic valve after a Stansel procedure and three patients with a regurgitant porcine valve in a left ventricular apex to descending aorta conduit. RESULTS: Placement of a double-umbrella device was attempted in seven of the eight patients and was successful in all seven. Device placement was not attempted in one patient because of the crescentic shape of his defect. Two patients required two devices for each closure; the other five required only one device each. Angiography, performed on six patients after device closure, demonstrated that three patients had a completely occluded defect, two had trivial residual flow and one patient had mild residual flow through the device. All significant complications occurred in one patient who had hemolysis and oliguria that resolved when the initial umbrella was replaced by a larger device. In addition, two devices migrated to the patient's pulmonary arteries but were retrieved in the catheterization laboratory without difficulty. No other early or late complications occurred in 21 to 50 months of follow-up. Of the four patients with a paravalvular leak, the one who did not receive a device died at operation, one patient died at operation for an associated defect (in the operating room the umbrella was found securely in place across the paraaortic defect) and two patients are clinically well at home after 21 and 32 months, respectively. Of the four patients with closure of a valvular leak, one patient remains well at home 50 months later, one patient died at operation for associated defects and two patients had additional successful surgical treatment and remain well 29 months after device placement. CONCLUSIONS: Transcatheter umbrella closure appears to be a reasonable alternative for closure of a valvular or paravalvular leak in patients who are poor operative candidates.

Aortic Valve↗

Transcatheter closure of coronary artery fistulas.

Transcatheter closure of a coronary artery fistula was undertaken in nine patients. There were three fistulas from the left circumflex coronary artery to the coronary sinus, three from the left anterior descending coronary artery to the right ventricular apex, two from the right coronary artery to the superior vena cava/right atrial junction and one fistula from the left circumflex artery to the pulmonary artery. The fistula was closed with Gianturco coils in six patients, a double-umbrella device in two and a combination of an umbrella and coils in one patient. All fistulas are completely occluded. Complications consisted of migration of two coils, one of which was retrieved, and a transient junctional tachycardia in one patient. In an additional three patients with multiple coronary artery fistulas, transcatheter occlusion was not attempted.

Adolescent↗

Effect of baffle fenestration on outcome of the modified Fontan operation.

BACKGROUND: The "fenestrated Fontan" (surgical baffle fenestration followed by transcatheter test occlusion and permanent closure after postoperative recovery) was adopted in an effort to reduce perioperative mortality and morbidity. This study assesses the effect of baffle fenestration on outcome. METHODS AND RESULTS: Patients having a modified Fontan operation with a cavocaval baffle and cavopulmonary anastomosis were retrospectively selected for study. Those with baffle fenestration (n = 91) were compared with those without baffle fenestration (n = 56) with respect to preoperative risk factors, age, anatomy, surgical date, and presence or absence of a previous bidirectional cavopulmonary anastomosis. Outcome variables were failure (death or take-down) and duration of postoperative pleural effusions and hospitalization. Survival and clinical status after hospital discharge were ascertained. The two groups did not appear to differ with respect to age or anatomic diagnosis. Patients having baffle fenestration were at significantly greater preoperative risk by univariate and multivariate analysis (p < 0.01). Operative failure was low in both groups (11% without and 7% with baffle fenestration, p = NS). Durations of pleural effusions and hospitalization were significantly shorter with baffle fenestration (p < 0.01). Neither date of surgery nor a previous bidirectional cavopulmonary anastomosis appeared to contribute to improved outcome. Patients with baffle fenestration had lower postoperative systemic venous pressure (p < 0.01). There were no late deaths. Functional status in both groups is good (82% in New York Heart Association class I). CONCLUSIONS: Baffle fenestration is associated with low mortality, significantly less pleural effusion, and significantly shorter hospitalization among high-risk patients having a modified Fontan operation.

Cardiac Surgical Procedures↗

Transcatheter closure of a large patent ductus arteriosus with the clamshell septal umbrella.

In 14 patients undergoing transcatheter closure of a large (greater than 4 mm diameter) patent ductus arteriosus, occlusion was attempted with use of the Bard Clamshell septal umbrella. Patient age ranged from 0.7 to 30.4 years. Isolated patent ductus arteriosus was present in 11 patients; 3 had additional congenital heart lesions. Moderate or severe pulmonary hypertension was present in four patients. The diameter of the patent ductus arteriosus ranged from 4.5 to 14 mm, as determined by contrast injection through an 11F sheath or by balloon sizing; it appeared larger by this method than by the standard angiographic method. All 14 patent ductus arteriosi were successfully closed. Prior embolization of a Rashkind umbrella was the reason for using a Clamshell device in three patients; one additional embolization of a Clamshell device occurred. All errant devices were retrieved at cardiac catheterization, without associated hemodynamic instability. No other complications occurred. Among the 14 patients, 11 had complete ductal closure by Doppler color flow mapping at last follow-up and 3 had trivial residual flow. All four patients having associated complex lesions or pulmonary hypertension, or both, had symptomatic improvement after the procedure, although one child (with Shone's anomaly) died 3 months later. The Clamshell device provides stable and effective closure of a large patent ductus arteriosus, and allows transcatheter closure to be offered to some patients who were previously considered unsuitable for this procedure.

Adolescent↗

Preoperative transcatheter closure of congenital muscular ventricular septal defects.

BACKGROUND: Surgical repair of muscular ventricular septal defects, particularly those associated with complex heart lesions carries a higher risk of reoperation and death than the repair of membranous defects. Closing a muscular defect through an incision in the systemic ventricle may cause late ventricular dysfunction. In a collaborative approach to this problem, we undertook preoperative transcatheter closure of muscular ventricular septal defects remote from the atrioventricular and semilunar valves, followed by the surgical repair of associated conditions. METHODS: In 12 patients selected jointly by a cardiologist and a cardiac surgeon, we attempted preoperative transcatheter umbrella closure of 21 defects. Half the patients had associated complex heart lesions; the others had had pulmonary-artery banding to reduce the amount of left-to-right shunting. Half had severe ventricular septal deficiency. RESULTS: All 21 defects were successfully closed without major complications. Subsequent cardiac surgery for associated conditions in 11 of the 12 patients resulted in a mean pulmonary-to-systemic flow ratio of 1.1, indicating minimal residual left-to-right shunting; 1 patient awaited surgical repair. No deaths, reoperations, or late complications have occurred after a follow-up of 7 to 20 months. CONCLUSIONS: A collaborative approach using transcatheter closure followed by the surgical repair of associated cardiac lesions may decrease rates of operative mortality, reoperation, and left ventricular dysfunction in patients with muscular ventricular septal defects.

Adult↗

Corrected transposition in situs inversus. Biventricular repair of associated cardiac anomalies.

Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.7 +/- 8.6 yr) underwent repair of associated cardiac anomalies, including ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) in all 13 patients and atrial septal defect (ASD) in 11. Systemic venous anomalies were present in four. Nine patients also had dextrocardia. Patches to close the VSD were placed on the right side of the ventricular septum (through a right [systemic] ventriculotomy) in two patients and on the left side of the ventricular septum in the other 11 via a left ventriculotomy in one or a morphological right (left-sided) atriotomy in 10. The POTO was relieved directly in five patients and bypassed by a left ventricle-pulmonary artery conduit in the other eight. There was one early death due to cerebral hemorrhage. Two patients required a pacemaker for permanent heart block. Other rhythm disturbances included transient heart block in three patients and late atrial fibrillation in one. All the survivors are asymptomatic as late as 15.5 years after the repair. One patient had conduit revision 10.5 years after the initial operation, two had residual POTO of greater than 40 mm Hg, and none had residual VSD. In conclusion, patients with [I,D,D] C-TGA present with a relatively uniform subset of anatomic lesions, including predominantly ASD, VSD, and POTO.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Vessel Prosthesis↗

Balloon angioplasty for congenital mitral stenosis.

We attempted balloon angioplasty in 9 children (ages 0.1 to 10 years) with congenital mitral stenosis. All were symptomatic with severe congestive heart failure and failure to thrive. Effective reduction in mitral gradient was initially achieved in 7 patients. For the entire group, mean valve gradient decreased from 14.8 +/- 5.0 to 8.1 +/- 6.7 mm (p = 0.0007) and mean valve area increased from 1.1 +/- 0.5 to 1.8 +/- 0.9 cm2/m2 (p = 0.003). More than mild mitral regurgitation developed in 2 patients but none required surgery for mitral regurgitation. Poor gradient relief followed dilation of valves with unbalanced chordal attachments, with restriction to the valve apparatus as in mitral arcade, and where the obstruction was not purely valvar as it is with a supramitral ring. No strokes, infection or deaths were due to the procedure. Based on these data, balloon angioplasty of congenital mitral stenosis should be considered before mitral valve replacement in younger patients and in those in whom mitral valve replacement would be problematic.

Angioplasty, Balloon↗

Long follow-up (to 43 years) of ventricular septal defect with audible aortic regurgitation.

From 1946 to March 1989, 92 patients (33 women and 59 men) were seen with ventricular septal defect (VSD) and audible aortic regurgitation (AR). The VSD was subcristal in 62 patients, subpulmonary in 21 and unknown in the remaining 9. The median age of onset of AR was 5.3 years. The risk of developing AR was 2.5 times greater in those with a subpulmonary VSD. The aortic valve was tricuspid in 90% and bicuspid in 10%. Prolapse was seen in 90% of those with subcristal VSD and in all with subpulmonary VSD. Pulmonary stenosis was seen in 46% of the patients with gradients ranging from 10 to 55 mm Hg. The incidence of infective endocarditis was 15 episodes/1,000 patient years. Among 20 patients followed medically, for 297 patient years, 1 died (1959) and most have been stable, including 2 followed for greater than 30 years. In the 72 patients operated on, there were 15 perioperative and 5 late deaths. Operations consisted of VSD closure alone in 7, VSD closure and valvuloplasty in 50 and VSD closure and aortic valve replacement in the other 15. Valvuloplasty was more effective in those operated on under age 10 compared to those older than 15 years (46 vs 14%). The durability of the valvuloplasty was 76% at 12 years and 51% at 18 years.

Actuarial Analysis↗

Early results and follow-up of balloon angioplasty for branch pulmonary artery stenoses.

Two hundred eighteen balloon angioplasty procedures were performed in 135 patients with branch pulmonary artery stenoses from June 1984 to February 1989. Arteries were dilated in patients with tetralogy of Fallot (n = 49), tetralogy of Fallot/pulmonary atresia (n = 64), isolated peripheral pulmonary artery stenoses (n = 58) and "other" lesions (the majority had truncus arteriosus or single ventricle and surgically induced pulmonary artery stenoses (n = 47). Mean age at dilation was 6.6 +/- 6.3 years (range 1 month to 38.5 years). The mean diameter of the lesion increased from 3.8 +/- 1.7 to 5.5 +/- 2.1 mm with dilation (p = 0.001). The overall success rate was 58% (127 of 218 dilations), assessed by the following criteria: an increase greater than or equal to 50% of predilation diameter, an increase greater than 20% in flow to the affected lung or a decrease greater than 20% in systolic right ventricular to aortic pressure ratio. Success did not correlate with patient age. Mean balloon to artery ratio was higher in successful (4.2) than in failed (3.0) angioplasty procedures (p = 0.0001). There were four early deaths: two of the patients had pulmonary artery rupture with angioplasty performed less than 1 month after pulmonary artery surgery. An aneurysm occurred in 11 arteries and transient pulmonary edema in four patients. At angiography performed a mean of 10 months (range 1 to 54) after dilation, the mean diameter of 57 arteries was unchanged (5.5 versus 5.4 mm). However, 5 of 32 initially successfully dilated vessels had returned to predilation size as a result of restenosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Double-umbrella closure of atrial defects. Initial clinical applications.

Forty patients were catheterized for closure of atrial septal defects with the Rashkind patent ductus arteriosus umbrella device, a modified Rashkind umbrella device, and the newly designed Lock Clamshell Occluder. Patients weighed 8 kg or more (a requirement for transvenous access with the 11F delivery sheath) and had defects suitable for closure based on two-dimensional echocardiography. The new device was at least 1.6 times the diameter of the atrial septal defect as determined by balloon sizing at catheterization. Patients were followed up by telephone, clinical examination, and echocardiography at 6 months. We attempted closure in 34 patients, with atrial septal defects ranging in diameter from 3 to 22 mm; device sizes ranged from 17 to 33 mm. Initial device position immediately after release was correct in all patients. A cerebral embolus occurred in one elderly patient before device placement--the patient died 1 week later. Two instances of early device embolization occurred, and devices were retrieved by catheter without complication. Follow-up of 31 patients discharged with devices in place, for a total of 31 patient-years, has yielded no umbrella-related complications. Adequate imaging studies in 19 patients 6.5 months after device placement revealed no atrial shunt in 12; residual flow through separate, previously unrecognized atrial septal defects occurred in two; and small residual leaks (less than 3 mm) around devices were present in five patients. This initial success indicates that double-umbrella closure of atrial septal defects will aid in the treatment of intracardiac defects.

Angiography↗

Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results.

Thirty-eight patients considered to be at increased risk for a Fontan repair underwent bidirectional cavopulmonary anastomosis. Twenty-one of the 38 had concurrent pulmonary artery reconstruction. Fontan risk factors included pulmonary artery distortion, elevated pulmonary artery resistance (greater than 2 Woods units) and/or pulmonary artery pressure (mean, greater than 18 mm Hg), atrioventricular valve regurgitation, systemic ventricular dysfunction, complex venous anatomy, and subaortic obstruction. There were no deaths, either early or late. Median arterial oxygen saturation increased from 79% to 84% (p less than 0.01). Median hospital stay was 8 days. No patient had pleural effusions after 7 days. Three patients had significant surgical complications. Five patients had inadequate relief of cyanosis; three of these had venous collaterals and two had severe ventricular dysfunction; the latter two patients subsequently had strokes. One of the patients with persistent cyanosis required a systemic-to-pulmonary artery shunt. We conclude that a bidirectional cavopulmonary shunt, with pulmonary artery reconstruction when indicated, provides adequate relief of cyanosis in most patients with single-ventricle lesions who are considered to be at increased risk for a Fontan repair. Relief of ventricular volume overload and pulmonary artery distortion may improve Fontan candidacy. Also, patients with persistent cyanosis after bidirectional cavopulmonary anastomosis should be catheterized for location and occlusion of venous collaterals. Further follow-up is necessary to determine the place of bidirectional cavopulmonary anastomosis in the management of patients at increased risk for a Fontan repair.

Anastomosis, Surgical↗