Congenital Cardiac Disease: A Review of 357 Cases Studied Pathologically

PEDIATRICS ◽  
1962 ◽  
Vol 30 (6) ◽  
pp. 874-874
Author(s):  
BRUCE D. GRAHAM

This book is a result of a combination of necropsy study of 357 cases of congenital cardiac disease undertaken at the Mayo Clinic between 1920 and 1954 and an extensive review of the literature on this subject. Inasmuch as this study is selective in nature, the authors specifically point out that the exact incidence in the general population, the sex distribution, causes of death, and frequency of occurrence of various complications cannot be determined without qualification.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3071-3071
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Y. Kristinsson ◽  
Therese M-L Andersson ◽  
Sandra Eloranta ◽  
Åsa Rangert Derolf ◽  
...  

Abstract Abstract 3071 Background: Available data on survival patterns among patients diagnosed with myeloproliferative neoplasms (MPN) show a great diversity. For instance, in essential thrombocythemia (ET) there are reports stating that survival is not affected by the disease while other investigators consider ET to be a serious disease that significantly reduces life expectancy. Patients with primary myelofibrosis (PMF) are consistently reported to have a shortened life span while polycythemia vera (PV) is associated with a reduced survival in many, but not all, studies. We conducted a comprehensive, population-based study to assess survival and to define causes of death MPN patients, and to compare patterns to the general population. Patients and Methods: The nationwide Swedish Cancer Registry was used to identify all cases of MPN between 1973 and 2008 with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios (EMRRs) were computed as measures of survival. The Cause of Death Registry was used to obtain information on causes of death both in the patient and the general population. Results: A total of 9,384 MPN patients were identified (PV n=4,389, ET n=2,559, PMF n=1,048 and MPN not otherwise specified (MPN NOS) n=1,288); 47% were males and the median age at diagnosis was 71. The reporting rate to the Cancer Registry increased over time being well above 95% during the most recent calendar period. There was a significant overall excess mortality in all subtypes of MPN, reflected in 5-year and 10-year RSRs of 0.83 (95% CI 0.81–0.84) and 0.64 (0.62-0.67) for PV, 0.80 (0.78-0.82) and 0.68 (0.64-0.71) for ET and 0.39 (0.35-0.43) and 0.21 (0.18-0.25) for PMF, respectively. Higher age at MPN diagnosis was associated with a poorer survival. For example, the 10-year RSR for patients <50 years was 0.86 (0.83-0.88) as compared to 0.35 (0.29-0.43; p<0.001) in those >80 years. Females had a superior survival, EMRR 0.72 (0.66-0.78), compared to males (reference 1.00). Survival of patients with MPN improved significantly over time with an EMRR of 0.60 (0.53-0.67) in 1983–1992, 0.29 (0.25-0.34) in 1993–2000 and 0.23 (0.19-0.27) 2001–2008 using the calendar period 1973–1982 as a reference (1.00). However, MPN patients of all subtypes including PV and ET had an inferior survival compared to the general population during all calendar periods indicating that these patients continue to experience higher mortality. The 10-year RSRs for patients diagnosed 1993–2008 were 0.72 (0.67-0.76) for PV and 0.83 (0.79-0.88) for ET (Figure). The most common causes of death in MPN patients were, in order, hematological malignancy 27.2%, cardiac disease 27.1%, solid tumors 12.4% and vascular events including thromboembolism and bleeding, 9.2%. Four per cent of patients in this cohort were reported to have died due to acute myeloid leukemia. Over time, the proportion of deaths due to cardiac disease and thromboembolic events has decreased. On the other hand, we observed an increasing relative number of deaths due to hematological malignancies during the more recent calendar periods. The relative risks of dying from these causes in relation to the general population will be presented. Summary/conclusion: In this large population based study including over 9,000 MPN patients, we found all MPN subtypes to have a significantly lower life expectancy compared to the general population. Survival improved over time, however patients of all subtypes including ET had an inferior relative survival even in the most recent calendar period. Especially during earlier years, a certain misclassification and under reporting of ET may have contributed to a reduction in survival rates in the ET group. The relative number of deaths due to cardiac disease and thromboembolic events decreased during more recent calendar periods. This, and the improvement in survival might reflect the introduction of better treatment strategies for both the disease itself and for the prevention and treatment of thromboembolic complications of MPNs. Disclosures: No relevant conflicts of interest to declare.


1996 ◽  
Vol 33 (4) ◽  
pp. 340-347 ◽  
Author(s):  
Rolf E. A. Nordström ◽  
Tuula Laatikainen ◽  
Tarja O. Juvonen ◽  
Redo E. Ranta

Extensive review of the literature since 1884 on cleft-twin sets yielded 364 cleft-twin sets. Of these, 118 were monozygotic (MZ) and 246 dizygotic (DZ) sets. In addition, Danish material on cleft twins, like our Finnish material, reflects the total number of clefts and cleft twins on a well-defined population during a well-defined time interval. Both sets of material also contain slightly over 100 pairs of twins. The Danish material and the literature review were compared to the Finnish material. The hospital records of all Finnish patients with operated clefts who were born between 1948 and 1987 were reviewed. Information was gathered regarding each patient, his parents, the pregnancy, and his twin or triplet siblings and other siblings. This search produced 105 sets of twins and three sets of triplets with clefts, 15 sets of twins being concordant regarding clefting. This resulted in a total of 120 cleft siblings, and the corrected cleft incidence of 1.72 promille, close to the overall cleft incidence rate in Finland between 1948 and 1975. Twinning was found to be associated neither with an increased nor with a decreased risk of clefting, and clefting could not be seen to increase twinning. Zygosity could be verified in 88 sets of twins; the total number of MZ sets was 17, and of DZ was 71, a 19% MZ rate. Although a higher incidence of clefting in MZ-twin sets has been proposed, no such higher or lower incidence could be found in our material. Recognized syndromes were found in 15 sets (14%), slightly higher than found in a large Finnish study on cleft probands (8.4%). Of these, three sets were monozygotic (MZ), all of them cleft palate (CP) and male sets, whereas eight sets were dizygotic (DZ). All 15 sets were CP only, with no one set with cleft lip and palate [CL(P)]. In our total Finnish-twin material of 105 sets, we found the CL(P)/CP ratio to be 39/66 (37%/63%). In all of the 120 affected siblings, the ratio was 35%/65%. The very high rate of 63% of CP twins is about two to three times higher than that reported in the literature of 364 sets where the CP ratio is 23%; compared to the Danish material with a CP ratio of 17%, it is almost four-fold. The overall CP rate in all clefts (not restricted to twins)in Finland compared to the rate In our neighboring Scandinavian countries was very much in line with this very big difference seen in the CP rate In our twin material. The CL(P) Incidence in our Finnish material is 0.61 promille and the CP incidence 1.11 promille. For MZCL(P), the incidence was 0.38 promilie; for MZCP, 0.91 promille; for DZCL(P), 0.52 promille, and for DZCP, 0.99 promille. Compared to the Danish figures, both the MZCP and DZCP incidence figures are nearly four-fold, with the MZCL(P) somewhat lower, and DZCL(P) less than half that number. The sex distribution of all cleft patients in our material was 44% male/56% female. Both in the Danish material and in the literature, it was the reverse. This difference is probably due mostly to the higher ratio of CP in the Finnish material. The CP group has a higher proportion of females in all these materials. The concordance (C) of the whole Finnish-twin material is 14%, compared to 16.5% reported in the literature and 8% for the Danish twins. The concordance for CL(P) In our material is many times lower (2.6%) than in the literature (17.1%) and In the Danish material (8%). In the Finnish twins, the C for CP is higher (17%) than that for the Danish (6%) and for the literature cleft-twin populations (14.3%). This is also true for the MZ and DZ subgroups. The heritability index (H) in CL(P) is lower for the Finns (17%) than for the Danish (45%) and for the literature materials (43%), and higher for CP (Finns 49%, Danish 33%, literature 36%). All of these data strongly suggest the quite different genetic behavior of both CL(P) and CP in Finland, with a much lower genetic component in the CL(P) and a higher In the CP.


2008 ◽  
Vol 18 (3) ◽  
pp. 307-310 ◽  
Author(s):  
Kirsten C. Odegard ◽  
Peter C. Laussen ◽  
David Zurakowski ◽  
Stephan J. Hornykewycz ◽  
Jonathan C. Laussen ◽  
...  

AbstractBackgroundCardiac anomalies are among the most frequent congenital malformations, but the basic underlying causes for most cardiac defects remains undetermined. Some 40 years ago, a higher incidence of blood group B was reported in a small number of African-American children with congenital cardiac defects. In this study, we sought to re-evaluate this association using a larger population.Methods and ResultsWe collected data from 1985 patients undergoing cardiac surgery from July, 2000, through December, 2004. We divided the patients into 6 subgroups according to their diagnosis. We then compared the prevalence of ABO phenotypes between the patients and the general population of the United States of America by chi-square analysis. There were no significant differences in the distribution of the ABO phenotypes amongst the subgroups of those with congenital cardiac disease, or any for subgroup compared to the general population.ConclusionWhile statistical significance is influenced by the size of the population within the United States of America and the small numbers within each of our subgroups of patients with congenital cardiac disease, we have been unable to show any relationship between the distribution of ABO phenotypes and the existence of congenital cardiac disease.


2020 ◽  
Vol 30 (Supplement_5) ◽  
Author(s):  
T M Mikkola ◽  
H Kautiainen ◽  
M Mänty ◽  
M B von Bonsdorff ◽  
T Kröger ◽  
...  

Abstract Purpose Mortality appears to be lower in family caregivers than in the general population. However, there is lack of knowledge whether the difference in mortality between family caregivers and the general population is dependent on age. The purpose of this study was to analyze all-cause mortality in relation to age in family caregivers and to study their cause-specific mortality using data from multiple Finnish national registers. Methods The data included all individuals, who received family caregiver's allowance in Finland in 2012 (n = 42 256, mean age 67 years, 71% women) and a control population matched for age, sex, and municipality of residence (n = 83 618). Information on dates and causes of death between 2012 and 2017 were obtained from the Finnish Causes of Death Register. Flexible parametric survival modeling and competing risk regression adjusted for socioeconomic status were used. Results The total follow-up time was 717 877 person-years. Family caregivers had lower all-cause mortality than the controls over the follow-up (8.1% vs. 11.6%) both among women (hazard ratio [HR]: 0.64, 95% CI: 0.61-0.68) and men (HR: 0.73, 95% CI: 0.70-0.77). Younger adult caregivers had equal or only slightly lower mortality than their controls, but after age 60, the difference increased markedly resulting in over 10% lower mortality in favor of the caregivers in the oldest age groups. Caregivers had lower mortality for all the causes of death studied, namely cardiovascular, cancer, neurological, external, respiratory, gastrointestinal and dementia than the controls. Of these, the lowest was the risk for dementia (subhazard ratio=0.29, 95%CI: 0.25-0.34). Conclusions Older family caregivers have lower mortality than the age-matched controls from the general population while younger caregivers have similar mortality to their peers. This age-dependent advantage in mortality is likely to reflect selection of healthier individuals into the family caregiver role. Key messages The difference in mortality between family caregivers and the age-matched general population varies considerably with age. Advantage in mortality observed in family caregiver studies is likely to reflect the selection of healthier individuals into the caregiver role, which underestimates the adverse effects of caregiving.


2008 ◽  
Vol 18 (S2) ◽  
pp. 256-264 ◽  
Author(s):  
Heather Dickerson ◽  
David S. Cooper ◽  
Paul A. Checchia ◽  
David P. Nelson

AbstractA complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval.The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the endocrine system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases.As surgical survival in children with congenital cardiac disease has improved in recent years, focus has necessarily shifted to reducing the morbidity of congenital cardiac malformations and their treatment. A comprehensive list of endocrinal complications is presented. This list is a component of a systems-based compendium of complications that will standardize terminology and thereby allow the study and quantification of morbidity in patients with congenital cardiac malformations. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, initiatives to improve quality, reporting of complications, and comparing strategies of treatment.


2013 ◽  
Vol 33 (10) ◽  
pp. 1457-1469 ◽  
Author(s):  
Kirsten E. Pijls ◽  
Daisy M. A. E. Jonkers ◽  
Elhaseen E. Elamin ◽  
Ad A. M. Masclee ◽  
Ger H. Koek

2000 ◽  
Vol 10 (6) ◽  
pp. 574-581 ◽  
Author(s):  
Christine Imms

AbstractFeeding an infant is an interactive process that facilitates social, emotional and culturally based skills. Children with congenital or acquired cardiac disease frequently require supportive regimes with regard to feeding so as to maintain weight, resulting in altered experiences for both the child and family. This study evaluated the practical, emotional and social ramifications for parents, of having a child with cardiac disease who also experienced difficulties with oral feeding. The study sampled three groups of parents who had children less than 3 years of age: those with cardiac disease who had difficulty in feeding, those with cardiac disease and no such difficulty, and those with no medical diagnosis. Parents completed a questionnaire about feeding, a time diary of activities involved in feeding, and Tuckman's Mood Thermometers, which measure anger and ‘poorness-of-mood’ associated with feeding the identified child. Parents of children with cardiac disease and a feeding difficulty reported a significantly more negative mood-state, and significantly longer time associated with feeding, than parents of children in the other two groups. Emerging themes from qualitative analysis of the data suggested that having a child with congenital cardiac disease producing difficulty in feeding had a strong negative impact on the whole family.


PEDIATRICS ◽  
1951 ◽  
Vol 7 (1) ◽  
pp. 151-151

This volume is a review of pertussis—its history, etiology, bacteriology, epidemiology, immunity, pathology, diagnostic problems and tests, clinical studies and treatment, based on an extensive review of the literature (706 references) and the clinical experience of the author.


1906 ◽  
Vol 52 (216) ◽  
pp. 92-108
Author(s):  
George Greene

It is the prevalent opinion that phthisis is the scourge of our English lunatic asylums, and that these institutions are, literally speaking, hotbeds for the growth and distribution of the tubercle bacillus. In the Irish asylums, where the death-rate from phthisis alone amounts to nearly 30 per cent. of all causes of death, there seems to be just grounds for this belief. In the English asylums, however, the mortality is much lower, and is but little, if any, greater than that amongst the general population. This can be verified by examination of the Registrar-General's Report, from which it appears that phthisis accounts approximately for one in twelve of all deaths. These figures probably represent less than the true proportion of deaths from phthisis, since post-mortem examinations in the majority of cases are not made, and thus, doubtless, many cases of pulmonary tuberculosis are overlooked.


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