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Biomedical subjects

J F Keane

Publications and source records attributed to J F Keane.

At least 37 records · Page 2Linked to original sources
PubMed1995

Anastomotic pseudoaneurysm of the ventricle after homograft placement in children.

A pseudoaneurysm of the right ventricle was diagnosed postoperatively in 8 patients between 1986 and 1992. One pseudoaneurysm formed after placement of a Carpentier-Edwards conduit and the other seven arose after placement of a homograft between the right ventricle and the pulmonary artery. Both aortic (n = 3) and pulmonary (n = 4) homografts had been used. In 6 patients, the homografts had been augmented proximally with Dacron, Gore-Tex, or pericardium. Pseudoaneurysms originated between the augmentation patch and the homograft in 4 patients, between the homograft or conduit and the myocardium in 3 patients (1 patient with and 2 without an augmentation patch), and between the patch and the myocardium in 1 patient. The prior operations performed were placement of a palliative conduit for tetralogy of Fallot with pulmonary atresia (n = 5), repair of truncus arteriosus (n = 2), and repair of absent pulmonary valve syndrome (n = 1). Pseudoaneurysms were discovered from 5 weeks to 4 years after the operation. Symptoms were present in 3 patients; in the others, diagnosis was made during follow-up on the basis of routine imaging studies. Symptoms, when present, were due to compression of surrounding mediastinal structures. Pseudoaneurysms ranged in diameter from 1.0 to 5.0 cm. Echocardiography and color-flow mapping reliably identified the pseudoaneurysm in the 6 patients in whom it was performed. Characteristic features included a well-defined, narrow aneurysm neck leading to an extracardiac echo-free space. Color-flow mapping demonstrated to-and-fro flow through the neck of the aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)

Aneurysm, False↗
PubMed1995

Intraaortic spring coil loops: early and late results.

OBJECTIVES: Our aim was to determine the late fate of intraaortic spring coil loops after embolization of aortopulmonary vessels. BACKGROUND: In some aortopulmonary collateral vessels and patent ductus arteriosi, the narrowest segment is close to the aorta; coils used to close such vessels will "straddle" the lesion, allowing one or more coil loops to protrude into the aortic lumen. The consequences of this procedure are unknown. METHODS: We reviewed the cineangiograms of all patients who had at least one aortopulmonary collateral vessel or patent ductus arteriosus closure between January 1, 1988 and August 31, 1993. From this group, 53 patients had multiple-plane angiographic evidence of intraaortic coil loops. All subsequent cineangiograms were reviewed to determine coil position or movement and evidence of recanalization or endothelial coverage of the coil loop. We also reviewed each hospital record or communicated directly with referring physicians to identify any subsequent complications such as emboli or endocarditis. RESULTS: Of the 53 patients with intraaortic coil loops, 49 patients had closure of one or more aortopulmonary collateral vessels (59 vessels), and 4 had closure of a patient ductus arteriosus (4 vessels). Patient follow-up ranged from 1 day to 66 months (median 20 months); follow-up was not available in 6 patients. Five of the 53 patients (9.3%; 95% confidence limits [CL] 3.1% to 20.7%) died at operation or of end-stage heart failure. Patients with late angiography had no residual flow in 31 of 35 aortopulmonary collateral vessels (88.6%; 95% CL 73.3% to 96.8%), and 0.5 mm separated the coil and aortic contrast column in all 12 coils with adequate angiography, suggesting endothelial coverage of the intraaortic coil loop. No episodes of stroke, embolic events, endocarditis or coil migration were reported. CONCLUSIONS: Although coil occlusion of aortopulmonary collateral vessels or patent ductus arteriosi may produce intraaortic coil loops, endothelialization appears routine. No late complications associated with intraaortic coil loops were observed.

Aorta, Thoracic↗
PubMed1995

Arrhythmias and intracardiac conduction after the arterial switch operation.

UNLABELLED: Intraatrial baffling procedures such as the Mustard or Senning repair of transposition of the great arteries have been associated with a high incidence of cardiac arrhythmias. These abnormalities are thought to arise from trauma to the sinus node and atrial muscle during the procedure. In the arterial switch operation, there is little intraatrial manipulation other than the repair of the atrial septal defect. In theory, rhythm disturbances after the arterial switch operation should be less prevalent. From January 1, 1983, to December 31, 1990, 390 patients (230 with intact ventricular septum and 160 with a coexisting ventricular septal defect) underwent an arterial switch operation. Electrocardiograms and 24-hour Holter monitor studies were obtained in the 364 survivors at hospital discharge and during follow-up. Limited intracardiac electrophysiologic studies were performed 6 to 12 months after the operation. RESULTS: Atrioventricular node function was preserved in most patients; seven patients (2%) had first-degree, two (0.7%) second-degree, and five (1.7%) had complete atrioventricular block (all with coexisting ventricular septal defect). All five patients with complete heart block received a permanent pacemaker. In those patients not having a permanent pacemaker, sinus rhythm was present in 96% on the surface electrocardiogram and 99% during 24-hour Holter monitor studies (1 month to 8.5 years, mean 2.1 years after the operation). Intracardiac electrophysiologic studies (n = 158) demonstrated normal corrected sinus node recovery times and AH intervals in 97% of patients. Atrial ectopy was present in 152 of 172 (81%) patients, with the majority (64%) of patients having only occasional premature beats without repetitive forms. Ventricular ectopy was a frequent finding during 24-hour monitoring. At hospital discharge 70% had ventricular ectopy; these values fell to 57% (in patients with intact ventricular septum) and 30% (in patients with a coexisting ventricular septal defect) at follow-up. In the early postoperative period, there were 25 episodes of supraventricular tachycardia (14 of which required therapy), 6 episodes of junctional ectopic tachycardia, and 9 episodes of ventricular tachycardia. The incidence of supraventricular tachycardia had fallen to 5% at follow-up, with no atrial flutter or fibrillation noted. Three patients had ventricular tachycardia on follow-up Holter studies. In summary, our results confirm the theoretical advantages of anatomic correction over atrial level correction of transposition of the great arteries with respect to preservation of sinus node function and low incidence of clinically significant tachyarrhythmias.

Arrhythmias, Cardiac↗
PubMed1994

Balloon aortic valvuloplasty in the young adult with congenital aortic stenosis.

Although balloon aortic valvuloplasty (BAV) is effective in the acute management of valvar aortic stenosis (AS), sustained benefit of this technique has been shown to vary in differing age groups. The role of BAV in the young adult with congenital, nondegenerative AS is poorly defined. The catheterization results and follow-up echocardiographic data were reviewed for all patients (n = 18) between the ages of 17 and 40 years (mean 23 +/- 7) undergoing BAV at our institution between March 1986 and January 1992. Peak-to-peak systolic ejection gradient was reduced by 55%, from 85 +/- 29 to 38 +/- 17 mm Hg (p < 0.001). Aortic valve area increased from 0.9 +/- 0.2 to 1.1 +/- 0.3 cm2 (p = 0.003). Results of dilation were inadequate in 2 patients. There were no deaths, myocardial infarction, or embolic events. The 16 "effectively" dilated patients were followed with serial echocardiography for a period of 1 to 82 months, demonstrating persistent gradient relief in most patients (maximal instantaneous gradient at follow-up, 55 +/- 17 vs 79 +/- 22 mm Hg before dilation, p < 0.001). Aortic valve replacements were performed in 5 patients, 2 with unsuccessful initial dilations. At the time of the most recent echocardiogram, 8 of 16 patients remained "incident free," with no subsequent catheterization or surgical interventions, a maximal instantaneous gradient of < or = 55 mm Hg, no more than moderate aortic regurgitation, and preserved ventricular function.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Dilation of intra-atrial baffle fenestrations: results in vivo and in vitro.

Spontaneous closure of intra-atrial baffle fenestrations occurred early postoperatively following a Fontan procedure in three patients (ages 0.8-2 yr) and were reopened using transvenous balloon dilation. Following this clinical experience with this complication we studied the effects of high pressure balloons of different sizes on fenestrations created in polytetrafluoroethylene (PTFE) patches. The intra-atrial baffle fenestration was found closed within hours after surgery in two patients and 32 days postoperatively in the other. Increasing edema, chest tube drainage, and renal dysfunction ensued rapidly. At catheterization the balloon dilation in two patients reduced the elevated right atrial pressure, edema, and pleural effusion and allowed an acceptable arterial saturation. In the other patient, with acidosis and a low cardiac output, transient cardiac arrest occurred following passage of the catheter through the fenestration and during later unsuccessful operation the fenestration, although open, was traversed by strands of clot. In the experimental study, a total of 45 fenestrations 4 mm in diameter were dilated with 5, 6, 7, 8, and 9 mm balloons. The fenestration areas remained increased significantly during the 14 days of observation in room air, with the larger balloons producing larger holes. Balloon dilation may be effective for spontaneous fenestration closure occurring in the early postoperative period after a fenestrated Fontan procedure. Our experimental data may be useful in selecting the balloon size in this setting.

Cardiac Surgical Procedures↗
PubMed1994

Use of cutoff pigtail catheters with intraluminal guidewires in interventional procedures in congenital heart disease.

Advances in interventional cardiac catheterization have necessitated technique modifications to ensure patient safety while allowing data acquisition. The use of cutoff pigtail catheters over guidewires for pressure recordings and angiography exemplifies such a modification. The primary purpose of this study was to ascertain contrast volume and flow rates achievable using such a system. A total of 140 used catheters of various sizes, with intraluminal guidewires (0.035 inch in 6, 7, and 8F; 0.025 inch in 5F) were subjected to contrast injections at increasing rates and volumes until rupture occurred. The 7F catheters allowed volume delivery at 15-40 ml at 15 ml/sec, 8F catheters 40 ml at 30 ml/sec, and 5 and 6F catheters 10 ml at 10 ml/sec. All ruptured within 3 cm of the hub, at < or = 900 PSI. A review of 737 clinical procedures revealed adequate pressure recordings and angiographic details in all, uncomplicated save for one intracardiac wire dislodgement.

Cardiac Catheterization↗
PubMed1994

Transesophageal echocardiographic guidance of transcatheter ventricular septal defect closure.

OBJECTIVES: This report describes transesophageal echocardiographic guidance of transcatheter closure of ventricular septal defects and its value as an adjunct to fluoroscopy and angiography in this procedure. BACKGROUND: Experience with transcatheter closure of ventricular septal defects has identified a diverse group of patients in whom it may be the procedure of choice. Although facilitating other interventional procedures, such as transcatheter closure of atrial septal defects, the value of transesophageal echocardiographic guidance for transcatheter ventricular septal defect closure has not been documented. METHODS: All patients who underwent ventricular septal defect closure with transesophageal echocardiographic guidance before November 1992 were included. Angiograms and echocardiograms were reviewed to evaluate device position and relation to valve tissue during placement and to assess residual flow after device implantation. The ability of transesophageal echocardiography to assess these variables was compared with fluoroscopy and angiography. RESULTS: Transesophageal echocardiographic guidance was used in 31 of the 83 catheterizations involving transcatheter ventricular septal defect closure performed between February 1990 and November 1992. Under transesophageal echocardiographic guidance, 45 devices were implanted: 23 in muscular ventricular septal defects, 17 in residual postoperative patch margin defects and 5 in other ventricular septal defects. Transesophageal echocardiographic guidance enhanced assessment of device position and proximity to valve structures and markedly improved assessment of residual flow. Assessment of residual flow with transesophageal echocardiography eliminated the need for multiple angiograms in some patients. Combining transesophageal echocardiography with fluoroscopy and angiography provided the most information. CONCLUSIONS: Transesophageal echocardiography facilitates transcatheter closure of ventricular septal defects by improving assessment of device position and effectiveness of closure. It is indicated when device placement is likely to be difficult or may interfere with valve structures or when multiple interventional procedures are anticipated.

Adolescent↗
PubMed1994

Severe congenital mitral stenosis in infants.

BACKGROUND: Despite current medical and surgical therapy, infants with symptomatic congenital mitral stenosis (CMS) continue to have high rates of morbidity and mortality. Catheter balloon dilation has been successful in relieving symptoms in a few older children with CMS but has not been evaluated in infants. METHODS AND RESULTS: We reviewed the records of 85 infants with CMS to assess severity of CMS, associated cardiac lesions, echocardiographic morphological appearance of the mitral valve, treatment, and outcome. There were five valve morphologies identified: "typical" hypoplastic mitral valve with symmetric papillary muscles (SYMM, 52%), supravalvar mitral ring (SVMR, 20%), double-orifice mitral valve (DOMV, 11%), hypoplastic mitral valve with asymmetric papillary muscles (ASYMM, 8%), and parachute mitral valve (PARA, 8%). Of the 85 infants, 31 (36%) were severely symptomatic, requiring intervention within the first 2 years. Balloon dilation was performed in 18 infants (age, 8.7 +/- 5.7 months; weight, 5.9 +/- 1.9 kg) and valve surgery in 13 (age, 10.9 +/- 5.9 months; weight, 6.7 +/- 2.1 kg). Balloon dilation decreased the peak transmitral gradient (LAa-LVED) > 30% in 15 of 18 initial attempts, from 20.3 +/- 8.2 to 10.9 +/- 4.9 mm Hg (P < .001), and the mitral valve area increased from 0.7 +/- 0.3 to 1.0 +/- 0.5 cm2/M2 (n = 10, P = .01). No infants died during the initial balloon dilation, although 2 of 3 died during a repeat procedure for restenosis. Other complications included significant mitral regurgitation in 7 of 18 patients (39%), 4 of whom had SVMR. Of the 18 infants, 8 (44%) had persistent symptomatic improvement at a mean follow-up of 14 months (range, 2 to 32 months). The 2-year survival after balloon dilation was 70%; 40% remained free of repeat intervention. Mitral valve surgery in 13 infants consisted of SVMR resections in 7, mitral valve replacements in 4, and LA-to-LV aortic valved homografts in 2. The operative mortality was 30%. Sustained improvement occurred in 8 (6 with SVMR) at 11 to 62 months of follow-up (mean, 30 months), with a 2-year survival of 60%. CONCLUSIONS: Infants with severe CMS have 2-year mortality rates approaching 40% regardless of treatment modality. Balloon dilation significantly reduces the transmitral gradient in the majority, but symptomatic improvement persists in only 40%. Procedure-related mortality was associated with repeat balloon dilation in patients with left ventricular hypoplasia. Balloon dilation of "typical" CMS can provide symptomatic relief in many infants, allowing postponement of valve replacement, although infants with SVMR do better with surgical management.

Cardiac Catheterization↗
PubMed1994

Surgical alternatives to the Fontan procedure incorporating a hypoplastic right ventricle.

BACKGROUND: Frequently the definitive operation for patients with a right ventricle (RV) that is too small to support full cardiac output is a modified Fontan operation. However, other surgical options exist that incorporate a small RV in the atriopulmonary pathway when biventricular repair is not feasible because of RV or tricuspid valve hypoplasia. The risks and benefits of these options have not been well defined. METHODS AND RESULTS: Between 1988 and 1993, 8 patients (6 with pulmonary atresia and intact ventricular septum and 2 with tricuspid valve stenosis and RV hypoplasia) underwent a cavopulmonary connection, which allowed right atrial blood to flow either to the pulmonary artery via the RV or directly via the cavopulmonary anastomosis. Age at surgery ranged from 1.5 to 9 years. The proximal right pulmonary artery was ligated in 5 patients, and the atrial septal defect was closed during the same procedure in 7 of the 8 patients. The echocardiographic right ventricular-left ventricular volume ratio ranged from 9% to 25%, and tricuspid valve z-scores ranged from 0 to -4. There were no deaths at a median follow-up of 24 months (range, 7 to 61 months). Mild exertional limitation was evident in only one patient. Postoperative echocardiograms demonstrated pulsatile systolic flow across the RV outflow tract in 5 patients and low-velocity diastolic-systolic flow in a sixth patient with extreme tricuspid valve hypoplasia. At postoperative cardiac catheterization (6 patients) right atrial mean pressures ranged from 7 to 13 mm Hg and mixed venous saturations from 62% to 70%. CONCLUSIONS: Right atrial decompression via a superior vena cava-to-pulmonary artery anastomosis allows incorporation of a small RV into the pulmonary circulation and closure of the atrial septum, with excellent results to date.

Cardiac Catheterization↗
PubMed1994

Unique features and prognosis of primum ASD presenting in the first year of life.

BACKGROUND: Primum atrial septal defect (ASD) is a defect that is usually associated with few symptoms and can be electively repaired with good results. A review of our experience with primum ASD identified a subset of patients characterized by presentation with congestive heart failure (CHF) in the first months of life and a requirement for early operation. Echocardiograms, catheterizations, operative reports, and clinical data were reviewed and compared with that on all other patients with primum ASD. Patients with transitional or complete atrioventricular (AV) canal defects were excluded. METHODS AND RESULTS: From January 1984 to December 1992, significant CHF was present in the first year of life in 11 patients (10.5%) with primum ASD who were managed surgically among 105 total patients undergoing repair of primum ASD. This early CHF group had a higher incidence of hypoplastic left-sided cardiac structures (9 of 11 patients) compared with other primum ASD patients (5 of 94, P < .001), including patients with coarctation (CoA) (n = 9), abnormal mitral valve (n = 7), left ventricular hypoplasia (n = 5), and subaortic stenosis (subAS) (n = 7). Other differentiators from patients without early CHF included incidence of Downs syndrome (0% versus 19%), elevated pulmonary artery pressures (72% versus 33% systemic, P < .001), earlier mean age at operation (8 months versus 5.4 years), greater incidence of reoperation after initial ASD closure (5 of 8 patients, 12 operations [5 subAS, 3 mitral valve, 3 CoA, 1 pacemaker] versus 4 of 93 patients, P < .001), and higher mortality (36% versus 1%, P < .001). CONCLUSIONS: Left-sided obstructive lesions must be sought in children with primum ASD presenting with CHF in the first year of life. The presence of these lesions alters prognosis and surgical management and mandates close follow-up, with particular attention to late appearance or progression of subaortic stenosis or deterioration of mitral valve function.

Child, Preschool↗
PubMed1993

Repair of double aortic arch associated with D-transposition of the great arteries.

D-transposition of the great arteries is infrequently associated with anomalies of the aortic arch. We report a rare case of double aortic arch associated with D-transposition of the great arteries. The diagnosis was made by angiography and transthoracic echocardiography. The patient underwent a successful simultaneous arterial switch operation and division of the vascular ring through a median sternotomy incision.

Aorta, Thoracic↗
PubMed1993

Intraoperative device closure of ventricular septal defects.

BACKGROUND: Surgical repair of muscular ventricular septal defects (VSDs) has been associated with significant morbidity and mortality when the defects are multiple, associated with complex cardiac lesions, or requiring left ventriculotomy. Transcatheter VSD closure may be difficult in patients weighing less than 7 kg or those with limited vascular access. We attempted intraoperative device closure of muscular VSDs during surgical repair of associated lesions. METHODS AND RESULTS: We considered intraoperative umbrella closure in 10 patients, 8 with additional complex heart lesions. "Swiss cheese" defects were present in 4. Median patient age was 4.7 months. Device closure was not attempted in 1 patient due to an inadequate septal rim. Nine umbrellas, 12 to 40 mm in diameter, were positioned to straddle the septum in all 9 patients. There were 3 early deaths, 2 in patients who were moribund preoperatively. The third was due to severe ventricular dysfunction. Among the 6 survivors, 3 had a Qp:Qs < or = 2 and right ventricular or pulmonary artery pressure less than half systemic. These patients had no further intervention. The other 3 had a Qp:Qs > 2. One died unexpectedly 5 weeks after cardiac surgery. The other 2 had subsequent closure of residual VSDs. All 5 survivors are well at follow-up of 8 to 25 months. CONCLUSIONS: Intraoperative umbrella VSD closure appears less successful than VSD closure in the catheterization laboratory. In selected patients, particularly very small infants, intraoperative device placement may be advantageous. Completeness of closure is difficult to assess intraoperatively.

Cardiac Catheterization↗
PubMed1993

Cardiorespiratory responses to exercise of patients with aortic stenosis, pulmonary stenosis, and ventricular septal defect.

BACKGROUND: The purpose of this study was to determine exercise tolerance and cardiac response to exercise for a large cohort of adult subjects with aortic stenosis, pulmonary stenosis, or ventricular septal defect participating in the Second Natural History Study of Congenital Heart Defects (NHS-2). METHODS AND RESULTS: Exercise testing was performed on 134 of 235 NHS-2 full participants with aortic stenosis, 195 of 331 NHS-2 full participants with pulmonary stenosis, and 324 of 594 NHS-2 full participants with ventricular septal defect. A Bruce treadmill exercise protocol was used. Mean exercise duration for patients with aortic stenosis, pulmonary stenosis, and ventricular septal defect was 86.5%, 94.2%, and 90.8% of predicted, respectively. For patients with aortic stenosis, there was a direct relation between echocardiographic Doppler maximum transaortic gradient and ST segment change during exercise. ST segment change during exercise was very uncommon for patients with pulmonary stenosis. For patients with ventricular septal defect, there was an association between arrhythmias noted during exercise and the presence of associated aortic insufficiency. CONCLUSIONS: Although exercise duration was well preserved for patients with each of the three defects, exercise tolerance was subnormal.

Adult↗
PubMed1993

Occurrence risk for congenital heart defects in relatives of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

BACKGROUND: Knowledge of the risk of occurrence of congenital heart defects in offspring of individuals with a congenital heart defect is important for genetic counseling and understanding the etiology of congenital heart diseases. METHODS AND RESULTS: A portion of the questionnaire mailed to all patients in the Second Natural History Study of Congenital Heart Defects (NHS-2) addressed marital status, pregnancy, miscarriage, and presence or absence of congenital heart defects or other congenital malformations in first-degree relatives. Offspring were not examined as a part of the study. For male probands with aortic stenosis (AS), three of 251 offspring had congenital heart disease, whereas one of 72 offspring of female probands with AS had congenital heart disease. For patients with pulmonary stenosis (PS), three of 176 offspring of male probands had congenital heart disease, whereas eight of 205 offspring of female probands had congenital heart disease. For male probands with ventricular septal defect (VSD), 10 of 334 offspring had congenital heart defects, and 11 of 384 offspring of female probands with VSD had offspring with congenital heart defects. The prevalence rate for noncardiac congenital anomalies in offspring of probands was 2%. CONCLUSIONS: Based on known congenital heart defects in offspring of probands in the NHS-2, occurrence rates of congenital heart disease in children of subjects with AS, PS, and VSD were 1.2% (confidence interval [CI], 0.34-3.1%), 2.8% (CI, 1.4-5.1%), and 2.9% (CI, 1.8-4.4%), respectively.

Adolescent↗
PubMed1993

Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

BACKGROUND: All of the 2,401 patients with aortic stenosis (AS), pulmonary stenosis (PS), or ventricular septal defect (VSD) admitted to the First Natural History Study of Congenital Heart Defects between 1958 and 1965 were eligible for the Second Natural History Study. Most patients with severe defects were managed surgically, and most with mild defects were managed medically. Final examination in the first study was carried out 8 years after admission. METHODS AND RESULTS: For AS, the incidence rate of bacterial endocarditis (BE) was 27.1 per 10,000 person-years. The incidence rate was 15.7 per 10,000 person-years for those managed medically and 40.9 per 10,000 person-years for those managed surgically. Most patients managed surgically had severe AS, and severity was more important to the risk of BE than the method of management. For PS, only one of the 592 patients with PS experienced BE. For VSD, the incidence rate of BE was 14.5 per 10,000 person-years. Size of the VSD was not associated with risk of BE. The risk of BE before closure of the VSD was more than twice that after surgery. CONCLUSIONS: The incidence rate of BE was nearly 35-fold the population-based rate. The increased incidence in patients with AS after valvotomy was a function of severity of the defect and not a function of surgery. Presence of aortic regurgitation in patients with AS did not increase the risk of developing BE. Surgical closure of VSD lowered the risk of BE.

Adolescent↗
PubMed1993

Second natural history study of congenital heart defects. Results of treatment of patients with aortic valvar stenosis.

BACKGROUND: From 1958 to 1969, 462 patients (mostly children) with aortic stenosis were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization. Most with gradients < 50 mm Hg were managed medically; most with gradients > or = 80 mm Hg had aortic valvotomy. Of those with gradients of 50-79 mm Hg, some were managed medically, and some were managed surgically. Most had a second cardiac 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 (the Second Natural History Study [NHS-2]) addresses the long-term results of medical and surgical management. METHODS AND RESULTS: Of the original cohort of 462 patients, 440 were alive at completion of NHS-1. New data were obtained on 371 (80.3%) of the original cohort. Probability of 25-year survival was 92.4% for those admitted with gradients < 50 mm Hg and 81.0% for those with admission gradients > or = 50 mm Hg. More than half of the cardiac deaths were sudden and unexpected. Forty percent of patients managed medically during NHS-1 subsequently required surgical management. Almost 40% of operated patients required a second operation. Three percent of the original cohort subsequently had bacterial endocarditis. There was a higher-than-normal prevalence of serious arrhythmias. Of NHS-2 full participants, 92.3% were in New York Heart Association functional class I. Most patients had low Doppler mean gradients. Clinically, 46.6% had aortic valve regurgitation. The final clinical status was excellent in 29.9%, good in 22.8%, fair in 28.6%, and poor in 18.7%. CONCLUSIONS: Patients with gradients < 25 mm Hg can be followed medically; however, progressive stenosis can occur, and approximately 20% will require intervention. If the gradient is > or = 50 mm Hg, there is a risk of serious arrhythmias and, possibly, sudden death. If the gradient is > or = 80 mm Hg, intervention is clearly indicated; as techniques improve, delaying intervention in patients with gradients of 50-79 mm Hg may not be advantageous. Patients with gradients of 25-49 mm Hg can be followed medically with annual evaluation.

Adult↗
PubMed1993

Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis.

BACKGROUND: From 1958 to 1969, 592 patients (mostly children) with pulmonary stenosis were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization. Most with gradients < 50 mm Hg were managed medically; most with gradients > or = 80 mm Hg had pulmonary valvotomy. Among these with gradients of 50-79 mm Hg, some were managed medically, and some were managed surgically. Most had a second cardiac catheterization at conclusion of NHS-1. More than 15 years had 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 the original cohort of 592 patients, 580 were alive at completion of NHS-1. New data were obtained on 464 (78.4%) of the original cohort. Probability of 25-year survival was 95.7%. Probability of survival was less (80%) in a subgroup of patients entering NHS-1 > 12 years old with cardiomegaly. Less than 20% of patients managed medically during NHS-1 subsequently required a valvotomy. Only 4% of operated patients required a second operation. Bacterial endocarditis occurred rarely. Ninety-seven percent were in New York Heart Association class I. There was a small but higher-than-normal prevalence of serious arrhythmias. Most patients, whether medically or surgically managed, had low Doppler maximum gradients. Final clinical status was excellent or good in 83%. CONCLUSIONS: Probability of survival is similar to that of the general population, and the vast majority of patients are asymptomatic. If valvotomy or valvuloplasty is required in a child, reoperation is rarely necessary. Patients with gradients < 25 mm Hg do not experience an increase in gradient. Patients with a gradient > or = 50 mm Hg should have valvotomy or valvuloplasty. Choice of management of patients with gradients of 40-49 mm Hg remains debatable.

Adult↗