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L Kidd

Publications and source records attributed to L Kidd.

At least 19 recordsLinked to original sources

Utilising handheld computers to monitor and support patients receiving chemotherapy: results of a UK-based feasibility study.

GOALS OF WORK: Recent changes in cancer service provision mean that many patients spend a limited time in hospital and therefore experience and must cope with and manage treatment-related side effects at home. Information technology can provide innovative solutions in promoting patient care through information provision, enhancing communication, monitoring treatment-related side effects and promoting self-care. PATIENTS AND METHODS: The aim of this feasibility study was to evaluate the acceptability of using handheld computers as a symptom assessment and management tool for patients receiving chemotherapy for cancer. A convenience sample of patients (n = 18) and health professionals (n = 9) at one Scottish cancer centre was recruited. Patients used the handheld computer to record and send daily symptom reports to the cancer centre and receive instant, tailored symptom management advice during two treatment cycles. Both patients' and health professionals' perceptions of the handheld computer system were evaluated at baseline and at the end of the project. MAIN RESULTS: Patients believed the handheld computer had improved their symptom management and felt comfortable in using it. The health professionals also found the handheld computer to be helpful in assessing and managing patients' symptoms. CONCLUSIONS: This project suggests that a handheld-computer-based symptom management tool is feasible and acceptable to both patients and health professionals in complementing the care of patients receiving chemotherapy.

Adult↗

The use of botulinum toxin type-B in the treatment of patients who have become unresponsive to botulinum toxin type-A -- initial experiences.

The increasing use of botulinum toxin type-A, especially for focal dystonia and spasticity has highlighted the issue of secondary non-responsiveness. Within the last few years botulinum toxin type-B (Myobloc/Neurobloc) has become commercially available as an alternative to type-A. This paper discusses our initial experience of botulinum toxin type-B in a total of 63 individuals who attended our botulinum clinic. Thirty-six patients had cervical dystonia and a secondary non-response to type-A toxin. Thirteen of these patients (36%) had a reasonable clinical response to Neurobloc and continue to have injections. The other 23 patients either had no response, or a poor response, or had unacceptable side effects and ceased treatment. A small number of people with blepharospasm, hemifacial spasm and foot dystonia also had a disappointing response to injection. Twenty patients with spasticity were also type-A resistant. Seven of these show some continuing response to type-B, without unacceptable side effects. These findings demonstrate that botulinum toxin type-B has a place in the management of patients who have become non-responsive to type-A, but overall the responses to type-B toxin were disappointing.

Antibodies↗

Image analysis of papillary thyroid carcinoma fine-needle aspirates: significant association between aneuploidy and death from disease.

BACKGROUND: Papillary thyroid carcinoma is the most common thyroid malignancy in the U.S. As many as half of patients with papillary carcinoma present with cervical lymph node metastases at the time of diagnosis. Metastatic disease involving cervical lymph node tissue has not historically been proven to correlate with a more aggressive course; however, distant metastases worsen prognosis. METHODS: Diagnostic fine-needle aspiration (FNA) smears from 26 primary and metastatic papillary carcinomas underwent Feulgen reaction and were studied by image analysis to determine DNA pattern, proliferation index, and the percentage of cells with DNA content >5C. The medical records of all the patients were reviewed for metastatic disease pattern and survival data. For metastatic pattern, two groups were defined: 1) confined to thyroid/local lymph node metastases/soft tissues of the neck involved by tumor, and 2) distant metastases. RESULTS: Among the 26 patients, 16 had "nonaggressive" DNA patterns described as diploid, abnormal diploid, or tetraploid, and 10 had "aggressive" DNA patterns described as aneuploid. Only 2 of the 16 patients in the "nonaggressive" DNA pattern group developed distant metastases, whereas 5 of the 10 patients in the aneuploid group developed distant metastatic disease. In addition, none of the 16 patients with "nonaggressive" DNA patterns died of disease, whereas 3 of the 10 individuals with DNA histograms interpreted as aneuploid did die of metastatic disease complications. CONCLUSIONS: Aneuploidy identified by image analysis of FNA of papillary thyroid carcinoma is significantly associated with death from papillary carcinoma (log rank test, P=0.027).

Adolescent↗

Urinary nuclear matrix protein 22 (NMP22): a diagnostic adjunct to urine cytologic examination for the detection of recurrent transitional-cell carcinoma of the bladder.

This study compares urine nuclear matrix protein 22 (NMP22) immunoassay and conventional urine cytologic examination for detecting recurrent transitional-cell carcinoma (TCC) of the urinary bladder. One hundred twenty-eight urine specimens from 107 patients with a history of TCC of the urinary bladder were studied. NMP22 immunoassay and conventional cytologic examination were performed on each specimen. The NMP22 and cytology results were then compared with the results of subsequent cystoscopies/surgical biopsies performed over a 6-mo follow-up period. The sensitivity of urine cytologic study for predicting recurrent TCC was 60%, while the sensitivity of NMP22 assay was 47%. When both NMP22 assay results and the cytologic interpretation were positive for TCC, the positive predictive value of the combined tests was 74%. When both tests showed negative results, the negative predictive power was 81%. Our findings suggest that urine NMP22 assay may represent a useful diagnostic adjunct to conventional urine cytologic examination for the detection of recurrent TCC of the urinary bladder.

Adult↗

Gross, histological and histomorphometric features of the navicular bone and related structures in the horse.

Forelimb navicular bones and associated soft tissues were collected from 3 groups of horses and subjected to pathological examinations. The groups consisted of 38 horses with clinical navicular disease (ND) and 2 control groups, with no history of forelimb lameness, consisting of 25 age-matched mature horses (A-MC) and 9 immature horses (IC). Histological and histomorphometric studies were performed on tissue samples from 10 ND, 10 A-MC and 5 IC horses. Gross changes seen only in ND horses included: full thickness defects in the palmar surface fibrocartilage, palmar cortex erosion, medullary lysis, flexor digitorum profundus tendon (FDPT) surface fibrillation, FDPT core lesions and adhesions between the FDPT and navicular bone. Palmar surface partial thickness fibrocartilage loss and distal border fragmentation were seen with a significantly greater incidence in ND than in A-MC and not observed in IC. Remodelling of the proximal border, FDPT surface colouration, palmar surface fibrocartilage colouration and proximal border entheseous bone were identified in ND and A-MC but not in IC. Mid-ridge synovial fossae and horizontal depressions in the palmar surface were identified in all groups. Histologically palmar fibrocartilage thinning and loss were associated with reduced palmar fibrocartilage cell density and chondrocyte cluster formation. Palmar fibrocartilage fibrillation, palmar cortical bone defects, fibromyxoid stromal change in the medulla, medullary pseudocyst formation and entheseous new bone formation were all seen in ND. The adjacent FDPT showed fibrillation, tag formation and degeneration of the dorsal surface. Necrotic foci were also present within the body of the tendon. Although not always present, medullary bone pseudocysts, separate mineralised foci and most changes on the dorsal surface of the FDPT were specific to ND. Bone histomorphometric parameters were compared among groups. Cross-sectional area reduced from the sagittal ridge to the medial and lateral margins of each navicular bone. IC navicular bones had a smaller subchondral area, subchondral bone volume and a greater osteoid volume than in the AC, indicating that these differences were age-related. In ND the medullary area was decreased but the trabecular bone volume increased. The palmar subchondral area was increased but contained bone with an increased porosity and osteoid volume. Changes occurred from the medial to the lateral margins of the bone in horses with ND indicating remodelling of the bony elements throughout the bone in ND. The histological and histomorphometric changes in the navicular bone and palmar fibrocartilage were considered similar of those found in articular hyaline cartilage and subchondral bone in osteoarthritis.

Animals↗

Clinical significance of negative and equivocal urinary bladder cytology alone and in combination with DNA image analysis and cystoscopy.

BACKGROUND: We evaluated the individual and combined ability of cytology (CYT), image analysis (IA), and cystoscopy (CYSTO) to predict the presence of transitional cell carcinoma (TCC) at 6 months of follow-up in patients with or without a prior history of urothelial carcinoma and negative (NEG) or equivocal (atypical or suspicious) urinary CYT. METHODS: Fifty-one patients (43 with prior TCC) provided 57 urinary samples that were evaluated by CYT and DNA IA. Forty-nine patients were evaluated by CYSTO. Disease status was reassessed at 6 months by a combination of clinical, CYSTO, CYT, and histologic follow-up. RESULTS: At 6 months' follow-up, the incidence of recurrence for patients with diploid, broad diploid, or aneuploid DNA histograms was 38%, 73%, and 100%, respectively. In the same group of patients, 43% of patients with NEG and "atypical" CYT recurred compared with 83.3% of patients with "suspicious" CYT. The predictive value (PV) of a positive (+) CYSTO evaluation was 100%; however, a NEG CYSTO examination was correct in only 73% of cases. Sensitivities of CYT, IA, and CYSTO to predict recurrence were 54%, 59%, and 62.5%, respectively, whereas the combined sensitivity of all three modalities was 72%. The +PV of combined CYT and IA in patients with prior TCC was 90% with aneuploidy 100% specific for malignancy; the NEG PV of combined CYT, IA, and CYSTO was 70%. CYT, IA, and CYSTO were highly significant in predicting recurrence (P = 0.0017, P = 0.0026, and P = 0.0002, respectively) whereas tumor grade and degree of invasiveness as assessed on initial biopsy were not significant. However, 11% of patients recurred between 6 months to 1 year who had NEG CYT, NEG CYSTO, and NEG IA. CONCLUSIONS: Diagnostic accuracy increases in patients with NEG or equivocal CYT if supplemented by DNA IA and CYSTO. In patients with no history of TCC, equivocal urine CYT and/or abnormal DNA IA can occur after chemotherapy, radiation therapy, or viral infection. In these patients, the combined approach together with accurate history is essential for correct diagnosis. For the small subpopulation of patients who recur but demonstrate no abnormalities on combined testing, more sensitive diagnostic tests, such as chromosomal abnormalities by in situ hybridization, need to be developed.

Adult↗

Cardinal clinical signs in the differentiation of heart murmurs in children.

OBJECTIVE: To determine the diagnostic accuracy of clinical assessment of heart murmurs in children and specific clinical features that are predictive of cardiac disease. DESIGN: Concurrent case series with pretest-posttest assessment. SETTING: Pediatric cardiology outpatient clinic. PARTICIPANTS: Five full-time academic pediatric cardiologists. MEASURES: For each of 222 consecutive patients who were seen for first-time evaluation of a heart murmur, the clinical findings and diagnostic impressions were recorded after clinical assessment. The results of electrocardiograms and echocardiograms were then reviewed, and changes in diagnostic impressions were recorded and compared with the original impressions. RESULTS: The prevalence of cardiac disease was 33%. Clinical assessment differentiated those patients with pathologic murmurs with a sensitivity of 92%, specificity of 94%, positive predictive value of 88%, and negative predictive value of 96%. If diagnostic uncertainty was considered an indication for echocardiography, then sensitivity and specificity increased to 97% and 98%, respectively. Missed disease included only trivial or minor lesions. Clinical features that were independently predictive of the presence of disease included murmurs that were pansystolic (odds ratio [OR], 54.0), grade 3 or more in intensity (OR, 4.84), heard best at the left upper sternal border (OR, 4.24) and harsh in quality (OR, 2.37), and the presence of an abnormal second heart sound (OR, 4.09) and an early or midsystolic click (OR, 8.35). CONCLUSIONS: Clinical assessment by a pediatric cardiologist is sufficient to distinguish pathologic from innocent heart murmurs. A genetic approach by using specific clinical features that are independently associated with disease may have some practical utility to noncardiologists.

Child↗

Image analysis of cellular DNA content in peritoneal fluid of patients with ovarian tumors of low malignant potential and invasive epithelial ovarian cancer.

Image analysis has rarely been used to quantitate the DNA content of intact cells derived from peritoneal fluid in patients with ovarian malignancy. An average of 118 (range 100-208) of the most atypical, visually selected Feulgen-stained cells in peritoneal fluid obtained from 46 patients undergoing primary cytoreductive surgery for histologically proven ovarian tumors of low malignant potential and truly invasive ovarian cancer were evaluated retrospectively using the SAMBA-4000 Image analysis system. The patients were stratified into 3 groups: 16 with ovarian tumors of low malignant potential (LMP), 14 with low-stage disease (LSD) (FIGO I and II), and 16 with advanced-stage (ASD) (FIGO III and IV). A pattern of high-degree aneuploidy with negative balance (means: LMP, 3.3; LSD, 20.5; ASD, 32.0), increased proliferative index (LMP, 11.2; LSD, 16.1; ASD, 13.9), and percentage of cells with DNA content greater than 5C (LMP, 6.7; LSD, 6.5; ASD, 9.5) was demonstrated in the peritoneal fluid of 8 of 16 patients with LMP (50%), 8 of 14 patients with LSD (57%), and 13 of 16 with ASD (81%). The median disease-free interval for patients with invasive epithelial ovarian cancer with peritoneal DNA diploid tumor cells was 57 months and for those with DNA aneuploid tumor cells 28 months, while in patients with LMP it was 65 and 54 months, respectively. In total, 19 patients developed a recurrence (LMP, 2; LSD, 5; ASD, 12) of which 17 were shown to have DNA aneuploid cells in the peritoneal fluid. Multivariate analysis, however, did not identify aneuploid population in the fluid, ploidy balance, proliferation indices, or degree of hyperploidy as an independently significant variable for predicting recurrence. It did appear, however, that tumor cells in peritoneal fluid with a degree of hyperploidy greater than 8 had a strong correlation for development of recurrence, although not statistically significant. Interactive image analysis of tumor cells in peritoneal fluid proved to be a valuable adjunct to cytodiagnosis. Seven of 28 patients (25%) who were underdiagnosed by cytology alone (LMP, 2; LSD, 3; ASD, 2) were shown to have malignant cells in their peritoneal fluid, while 2 of 18 patients (11%) who were called positive by cytology (LMP, 1; LSD, 1) showed diploid pattern histograms and upon review were interpreted as reactive mesothelial cells.

Adult↗

DNA image analysis of urinary cytology: prediction of recurrent transitional cell carcinoma.

To evaluate the utility of image analysis in monitoring patients with transitional cell carcinoma, we studied, by cytologic means and by image analysis, 78 urinary tract specimens from 66 patients, of whom 49 (74%) had a previous history of transitional cell carcinoma. The specimens consisted of 51 (65%) voided urine specimens, 12 (15%) bladder washings, 8 (10%) ureteral washings, 3 (4%) ureteral brushings, 2 (3%) renal pelvic washings, and 2 (3%) catheterized urine specimens. DNA histograms were classified into five patterns on the basis of their DNA index and the percentage of their cells with DNA content greater than 5c: diploid (single peak in the 2c region with no cells greater than 5c), intermediate (diploid with less than 10% of cells greater than 5c), aneuploid (single peak or multiple peaks between the 2c and 4c region or more than 10% of cells greater than 5c), tetraploid (at least 10% of cells in the 4c region and a corresponding peak at 8c), and polyploid (multiple peaks in the 2c, 4c, 8c, and 10c regions). Of the 78 cases, 22 were diploid, 24 were intermediate, 29 were aneuploid, one was tetraploid, and two were polyploid. Histologic confirmation or clinical follow-up was found in 29 aneuploid cases, 13 intermediate cases, and one diploid case. Most cases of carcinoma in situ (five of six) and invasive tumors (12 of 17) were aneuploid. The sensitivity was 100%, and the specificity was 73% when cytologic and image analysis results were combined. We conclude that image analysis, when combined with cytologic examination, is a reliable noninvasive diagnostic test for monitoring patients with transitional cell carcinoma; aneuploidy is specific for malignancy; and the presence of cells greater than 5c, although frequently associated with tumor recurrence, can be seen in non-neoplastic conditions.

Adult↗

An evaluation of parental concerns and misperceptions about heart murmurs.

This study sought to determine the extent of anxiety and misperceptions about heart murmurs for consenting parents of 182 children referred for first-time pediatric cardiology assessment (including echocardiography) of a heart murmur. From questionnaires completed before assessment, 22% of parents indicated that they were extremely concerned, and only 16% could define a heart murmur as a sound made by the heart. From 1-month follow-up questionnaires obtained from parents of children without heart disease at assessment, 10% continued to believe that their child had a heart problem. Cardiology assessment may not provide complete reassurance to all families and additional interventions may be necessary.

Adolescent↗

Linear single-dose pharmacokinetics of ganciclovir in newborns with congenital cytomegalovirus infections. NIAID Collaborative Antiviral Study Group.

The pharmacokinetic characteristics of ganciclovir were determined in neonates (age range, 2 to 49 days) after an 1-hour intravenous infusion of a single dose of either 4 mg/kg (n = 14) or 6 mg/kg (n = 13). Twenty-seven newborns with symptomatic cytomegalovirus inclusion disease were enrolled in this open phase I-II pharmacokinetics, safety, and tolerance trial of ganciclovir at one of two doses. Ganciclovir disposition was best described by a one-compartment open model with zero-order input and first-order elimination. The mean elimination half-life (t1/2) for both dose groups was 2.4 hours. The mean apparent volume of distribution (Vd) was 669 +/- 70 ml/kg for the 4 mg/kg group and 749 +/- 59 ml/kg for the 6 mg/kg group. The mean total body clearance (CL) for the 4 mg/kg and 6 mg/kg groups was 189 +/- 28 ml/hr/kg and 213 +/- 21 ml/hr/kg, respectively. No significant differences were observed in Vd or CL between the two groups. The Vd, expressed in milliliters, increased with increasing patient weight (r = 0.689; p = 0.0001). The CL, expressed in milliliters per hour per kilogram, increased with increasing age (r = 0.413; p = 0.032). No significant differences were observed between the two dose groups for the area under the curve normalized for dose (AUC/Dose) or the maximum plasma concentration normalized for dose (Cmax/Dose), indicating that ganciclovir exhibited linear pharmacokinetics in these neonates.

Creatinine↗

A comparison of responses to analgesia of the navicular bursa and intra-articular analgesia of the distal interphalangeal joint in 59 horses.

Analgesia of the distal interphalangeal (DIP) joint and of the navicular bursa was performed independently in the lame or lamer forelimb of 59 horses, in which lameness was significantly improved by perineural analgesia of either the palmar digital or palmar (abaxial sesamoid) nerves. In 3 horses no improvement was detected. Lameness was improved by analgesia of the DIP joint but there was no response to analgesia of the navicular bursa in 3 horses. In 12 horses, lameness was improved by analgesia of the navicular bursa but intra-articular analgesia resulted in no change. Forty-one horses responded both to analgesia of the navicular bursa and intra-articular analgesia of the DIP joint. Synovial fluid was retrieved from all DIP joints but from only one navicular bursa. It is suggested that a positive response to analgesia of the DIP joint does not necessarily imply that pain arises from the joint per se, but a positive response to intra-bursal analgesia probably reflects pathology of the bursa per se, the navicular bone and/or supporting ligaments and/or the DDFT. Radiographic abnormalities of the navicular bone in the lame or lamer limb were detected in 35 horses, all of which showed some response to analgesia of the navicular bursa. In 25 of these horses (71%) changes were only identifiable in the palmaroproximal-palmarodistal oblique views.(ABSTRACT TRUNCATED AT 250 WORDS)

Analgesia↗

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↗

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↗

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↗

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↗

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↗