Aortic atresia: survival to adulthood without surgery.

Aortic atresia is a rare congenital cardiac defect. It usually leads to death in the neonatal period. A patient with aortic atresia has survived to the age of 24 years without any surgical procedure. In view of the uncertain results of reconstructive surgery this case may have therapeutic implications.     

Endoluminal closure of ductus arteriosus. A cooperative study

Thirty-two patients from 3 Pediatric Cardiological Centres underwent attempted endoluminal transcatheter closure of a patent ductus arteriosus. The patients' age ranged from 8 months to 67 years, but there were only 2 adults (18 and 67 years) in this series. All patients had a murmur which was continuous in 28 cases and systolic in 4 cases. Nine patients had left ventricular hypertrophy on the electrocardiogram and radiological cardiomegaly. Closure of the patent ductus was attempted by Rashkind's technique using a double umbrella of 12 mm diameter for ductus less than 4 mm diameter (26 cases), and an umbrella of 17 mm diameter when the ductus was wider (6 cases). The immediate results judged by retrograde aortography showed total occlusion in 12 patients and incomplete closure in 17 others with persistence of a minimal left-to-right shunt. In 3 cases, embolisation of the umbrella into the left pulmonary artery (2 cases) or right pulmonary artery (1 case), required thoracotomy to extract the umbrella and at the same time section-suture of the ductus arteriosus. Color Doppler echocardiographic follow-up showed the disappearance of the residual shunt in seven children.     

Transcatheter Versus Rapid-Deployment Aortic Valve Replacement: A Propensity-Matched Analysis From the German Aortic Valve Registry

ObjectivesThis study sought to compare patient characteristics, procedural outcomes, and valve hemodynamics of surgical aortic valve replacement (SAVR) with current-generation rapid-deployment valves (RDVs) versus transcatheter aortic valve replacement (TAVR) with current-generation transcatheter heart valves (THVs).BackgroundThe patient population currently treated with RDVs may have potential similarities with the current TAVR population, but comparative studies in a large patient population remain scarce.MethodsA total of 16,473 patients who underwent isolated SAVR using current-generation RDVs or isolated transfemoral TAVR with current-generation THVs between 2011 and 2017 were enrolled into the German Aortic Valve Registry. Baseline, procedural, and in-hospital outcome parameters were analyzed for RDVs and THVs before and after 1:1 propensity score matching. Furthermore, RDVs and THVs with similar design characteristics were compared with each other.ResultsA total of 1,743 patients received SAVR with an RDV, whereas 14,730 patients were treated with transfemoral TAVR. Patients treated with TAVR were significantly older and had higher surgical risk scores. Following valve replacement, patients treated with an RDV had a significantly higher rate of disabling stroke (1.7% vs. 1.1%; p = 0.03), need for transfusion of >4 red blood cell units (8.5% vs. 1.4%; p < 0.001), and new onset renal replacement therapy (1.9% vs. 1.2%; p = 0.01), whereas the need for a new permanent pacemaker was lower (8.4% vs. 14.9%; p < 0.001). In-hospital mortality was similar (1.6% vs. 1.8%; p = 0.62). These findings persisted after 1:1 propensity score matching, but in-hospital mortality was significantly higher after RDVs (1.7% vs. 0.6%; p = 0.003). Balloon-expandable (BE) RDVs had significantly lower residual gradients compared with BE-THVs, while self-expanding (SE)-RDVs had significantly higher residual gradients compared with SE-THVs.ConclusionsIn a large all-comers’ registry, TAVR with current-generation THVs was associated with improved in-hospital outcomes compared with SAVR with current-generation RDVs. The pacemaker rate is significantly higher with TAVR. Post-procedural hemodynamic function varied between individual RDVs and THVs.     

Sarcoidosis in internal medicine. A cooperative study of 554 cases

The authors report the results of a retrospective cooperative study of 554 cases of sarcoidosis admitted to departments of internal medicine. The condition is more common in women (57 p. 100) than men (43 p. 100), but the onset is earlier in men. The clinical presentation is very variable and includes a wide variety of extra-thoracic localisations. "Guided" biopsy of early accessible lesions (skin, subcutaneous lymph nodes) is positive in 92 p. 100 of cases: the sensitivity of "blind" biopsy is less impressive, ranging from 49 to 69 p. 100 according to the tissue biopsied (bronchial mucosa, accessory salivary glands, liver). Alveolar lavage is positive in 65 p. 100, and angiotensin converting enzyme is positive in 58 p. 100 of untreated cases. There is a clear correlation between the results of these investigations and the radiological stage of the mediastino -pulmonary disease. Steroid therapy was instituted in 68 p. 100 of cases. The outcome with or without therapy was favourable in 82 p. 100 of cases, confirming the usually benign course of sarcoidosis when the heart is not involved.     

Sudden unexpected death from cardiovascular disease in children. A cooperative international study

The results of an international cooperative study of sudden unexpected death from cardiovascular disease in children are reported. Sudden natural death was defined as death occurring instantaneously or within 24 hours of the onset of acute symptoms or signs in ambulatory, nonhospitalized children aged 1 to 21 years. Twenty institutions representing 10 countries contributed a total of 254 acceptable case reports. In 186 cases the child had had no cardiac surgery. Four heart conditions accounted for 53 percent of these “medical” deaths: congenital aortic stenosis (18 percent), Eisenmenger's syndrome (15 percent), cyanotic congenital heart disease with pulmonary stenosis or atresia (10 percent) and hypertrophic obstructive cardiomyopathy (9 percent). A total of 33 types of cardiovascular disease were reported. Sixty-eight chlldren died months or years after cardiac surgery. In 28 of these 68 patients death appeared to be causally related to the preceding surgery rather than to the underlying cardiovascular disease. All but 2 of the 28 probably died of an arrhythmia, usually without warning.The period between the onset of acute signs and symptoms (terminal episode) was less than an hour (usually instantaneous) in 73 percent of all 254 patients. In 95 percent of patients in the “medical” group heart disease was known or suspected, but a majority of these patients were asymptomatic. Warning syncope occurred in only 16 percent. A majority of the patients (58 percent) were inactive at the time of the terminal episode (Standing, sitting, lying or sleeping). Only 10 percent were engaged in active sports.This large study appears to establish better than previous data the relative frequency of sudden unexpected death among children with heart disease in Western Europe and Central and North America. Surgical and medical preventive measures, and the indications for engaging in sports, are discussed.     

Percutaneous angioplasty of branch pulmonary artery stenosis. A cooperative study]

Fifty balloon angioplasties of branch pulmonary artery stenosis in 34 patients aged 4 months to 20 years, performed in 7 French Centres, were included in this study: they were performed from 1984 to 1991 and concerned severe stenoses which were congenital in 36 cases and secondary to surgery in 14 cases. The criteria of inclusion were: diameter of stenosis less than or equal to 8 mm, right ventricular systolic pressure (RVP) greater than or equal to 50 mmHg, RVP/aortic pressure ratio (RVP/AO) greater than or equal to 50%, and a significant perfusion defect on radionuclide angioscintigraphy. There were no operative complications. The diameter of the stenosis increased by greater than 40% in 23 cases (46%); in only 7 of these cases (14%) did the RVP and RVP/AO ratio decrease by more than 20%, the RVP being less than 50 mmHg, or did the perfusion scintigraphy improve. No cases of restenosis were observed. The reasons for failure are discussed together with the limitations of this study. The respective indications of surgery and angioplasty are not easy to determine for these complicated lesions. New techniques such as the use of stents should improve results.     

Improved treatment results in childhood acute myelogenous leukemia: a report of the German cooperative study AML-BFM-78

One hundred fifty-one children with acute myelogenous leukemia (AML) entered the cooperative study BFM-78 between December 1978 and October 1982. The initial therapy consisted of an intensive induction and consolidation regimen over eight weeks with seven different drugs and cranial irradiation. It was followed by maintenance with thioguanine and cytosine arabinoside for two years and additional Adriamycin during the first year. One hundred nineteen (79%) patients achieved a complete remission. Thirteen (9%) children died of early hemorrhages. After a median follow-up time of 36 (12 to 57) months, 47 relapses have occurred, with CNS involvement in seven cases. The life table analysis revealed a probability for overall survival after almost five years of 45% (SD, 4%), for event-free survival 41% (SD, 4%), and for the event- free interval 52% (SD, 5%). Up to now, no relapse was seen after 2 1/2 years. Risk factor analysis showed that early fatal hemorrhages occurred predominantly in children with M5 FAB type and with initial leukocytosis. An initial high WBC count and liver enlargement were unfavorable parameters for achieving remission. No factors could be identified concerning the risk for relapse. These data indicate that the applied treatment strategy is successful in inducing complete remissions in about three fourths of children with AML and also in enhancing considerably the chances for long-term remission.     

Carrier detection in hemophilia A: a cooperative international study. I. The carrier phenotype

Eight laboratories in six countries cooperated to clarify several issues concerning the phenotypes of heterozygous carriers of hemophilia "A." Plasma levels of factor VIII (F.VIII:C, formerly VIII:C) and von Willebrand factor (VWF:Ag, formerly VIIIR:Ag) of carriers and normal women were determined by various "in-house" methods; a single lyophilized plasma standard was used for all assays. Analysis of the collated data from 336 carriers (296 obligatory carriers and 40 sporadic carriers) and 137 normal women showed that there was no difference in the F.VIII:C levels of "paternal" carriers (women who had obtained the abnormal gene from their fathers) and "maternal" carriers. Neither was there a difference in the VWF:Ag levels of normal women and either type of carrier. Age was found to have a significant effect on both F.VIII:C and VWF:Ag, values being higher at very young and very old ages, the minima occurring in the 25- to 30-year range. ABO blood type had a striking effect. Women of types A, B, and AB (designated non- O in the study), both normals and carriers, had significantly higher levels of both factors than did women of type O. Analysis by laboratories showed that differences in mean levels of both factors between laboratories were highly significant. It was concluded that age, ABO blood type, and laboratory variation should be taken into account in carrier detection.     

Aortic atherosclerosis in La?nnec's cirrhosis. A comparative study using a planimeter

A postmortem comparison of atherosclerotic lesions of the aorta was made between 107 cirrhotic and 168 control subjects with mean ages of 61 and 64, respectively. The cause of death in 93 of the control subjects was malignant neoplasms. Individuals with a history of hypercholesterolemia, diabetes, hypertension, ischemic arterial disease, cerebral vascular accidents, or myocardial infarction were eliminated from this study. The entire aorta was removed during necropsy, and the ratio between the total aortic surface and the surface areas of fibrous, calcified, and ulcerated lesions was determined, using a planimeter. Statistical analysis disclosed no difference between the cirrhotic or control groups in either the extent or type of atherosclerotic lesions.     

The German Aortic Valve Registry (GARY): A Nationwide Registry for Patients Undergoing Invasive Therapy for Severe Aortic Valve Stenosis

Background The increasing prevalence of severe aortic valve defects correlates with the increase of life expectancy. For decades, surgical aortic valve replacement (AVR), under the use of extracorporeal circulation, has been the gold standard for treatment of severe aortic valve diseases. In Germany ~12,000 patients receive isolated aortic valve surgery per year. For some time, percutaneous balloon valvuloplasty has been used as a palliative therapeutic option for very few patients. Currently, alternatives for the established surgical procedures such as transcatheter aortic valve implantation (TAVI) have become available, but there are only limited data from randomized studies or low-volume registries concerning long-time outcome. In Germany, the implementation of this new technology into hospital care increased rapidly in the past few years. Therefore, the German Aortic Valve Registry (GARY) was founded in July 2010 including all available therapeutic options and providing data from a large quantity of patients.Methods The GARY is assembled as a complete survey for all invasive therapies in patients with relevant aortic valve diseases. It evaluates the new therapeutic options and compares them to surgical AVR. The model for data acquisition is based on three data sources: source I, the mandatory German database for external performance measurement; source II, a specific registry dataset; and source III, a follow-up data sheet (generated by phone interview). Various procedures will be compared concerning observed complications, mortality, and quality of life up to 5 years after the initial procedure. Furthermore, the registry will enable a compilation of evidence-based indication criteria and, in addition, also a comparison of all approved operative procedures, such as Ross or David procedures, and the use of different mechanical or biological aortic valve prostheses.Results Since the launch of data acquisition in July 2010, almost all institutions performing aortic valve procedures in Germany joined the registry. By now, 91 sites which perform TAVI in Germany participate and more than 15,000 datasets are already in the registry.Conclusion The implementation of new or innovative medical therapies needs supervision under the conditions of a well-structured scientific project. Up to now relevant data for implementation of TAVI and long-term results are missing. In contrast to randomized controlled trials, GARY is a prospective, controlled, 5-year observational multicenter registry, and a real world investigation with only one exclusion criterion, the absence of patients' written consent.     

Phenylketonuria in adulthood: a collaborative study

During 1967–1983, the Maternal and Child Health Division of the Public Health Services funded a collaborative study of 211 newborn infants identified on newborn screening as having phenylketonuria (PKU). Subsequently, financial support was provided by the National Institute of Child Health and Human Development (NICHD). The infants were treated with a phenylalanine (Phe)-restricted diet to age 6 years and then randomized either to continue the diet or to discontinue dietary treatment altogether. One hundred and twenty-five of the 211 children were then followed until 10 years of age. In 1998, NICHD scheduled a Consensus Development Conference on Phenylketonuria and initiated a study to follow up the participants from the original Collaborative Study to evaluate their present medical, nutritional, psychological, and socioeconomic status.Fourteen of the original clinics (1967–1983) participated in the Follow-up Study effort. Each clinic director was provided with a list of PKU subjects who had completed the original study (1967–1983), and was asked to evaluate as many as possible using a uniform protocol and data collection forms. In a subset of cases, magnetic resonance imaging and spectroscopy (MRI/MRS) were performed to study brain Phe concentrations.The medical evaluations revealed that the subjects who maintained a phenylalanine-restricted diet reported fewer problems than the diet discontinuers, who had an increased rate of eczema, asthma, mental disorders, headache, hyperactivity and hypoactivity. Psychological data showed that lower intellectual and achievement test scores were associated with dietary discontinuation and with higher childhood and adult blood Phe concentrations. Abnormal MRI results were associated with higher brain Phe concentrations. Early dietary discontinuation for subjects with PKU is associated with poorer outcomes not only in intellectual ability, but also in achievement test scores and increased rates of medical and behavioural problems.     

Quantitative analysis of surface P-wave morphology in isthmus ablation for type 1 atrial flutter: differentiation between complete isthmus block and slow isthmus conduction.

Changes in P-wave morphology in inferior leads during atrial pacing at the margins of the carvo-tricuspid isthmus have been reported to be useful for predicting the creation of isthmus block in radiofrequency (RF) ablation of type I atrial flutter (AFL). However, it is not known whether these changes in P-wave morphology allow the clinician to differentiate between complete isthmus block and slow isthmus conduction. P-wave morphology during low lateral right atrial (LLRA) pacing, as well as during coronary sinus ostium (PCS) pacing, was evaluated prior to ablation, during slow isthmus conduction, and after complete isthmus block in 30 patients with AFL. Changes in P-wave morphology during LLRA pacing were not sufficient to differentiate between complete isthmus block and slow isthmus conduction. While changes in P-wave morphology in lead II from inverted to biphasic during PCS pacing were observed in both slow isthmus conduction and complete isthmus block, the ratio of the positive component to the total P-wave amplitude (P-wave ratio) was significantly different between slow isthmus conduction (20+/-17%) and complete isthmus block (40+/-11%) (P<0.0001). When the P-wave ratio in lead II during PCS pacing was more than 75% of the F-wave ratio in lead II during AFL, bilateral complete isthmus block was predicted with a sensitivity of 88%, a specificity of 71%, a positive predictive value of 75%, and a negative predictive value of 85%. These results indicate that a P-wave ratio greater than 20% or a P-wave ratio during PCS pacing greater than 75% of the F-wave ratio during AFL may predict a bidirectional complete isthmus block.     

The treatment of chronic stable angina with isradipine. A cooperative Latin American study

In order to study the efficacy and tolerance of isradipine, a new Ca++ antagonist for the treatment of stable chronic angina, a multicentric cooperative study was carried out in eight Latin American countries (Argentine, Chile, Colombia, Ecuador, Mexico, Peru, Uruguay and Venezuela), which included 169 patients (60% men and 40% women), average age 62.6 +/- 9.7. Patients with more than 4 biweekly anginal crisis were accepted, with one or more of the following inclusion criteria: coronariographic evidence of obstruction greater than 60% in one or more vessels, IAM history, positive scintigraphy and positive effort test. The trial was single-blind, with placebo during the admission phase (2 weeks) and active treatment for 12 weeks. isradipine was administered in increasing doses of 2.5, 5, and 7 mg thrice a day, according to the presence or absence of anginal crisis. It was observed that the average frequency of weekly pains decreased from 8.2 +/- 7 under placebo to 6.3 +/- 7.5 under isradipine at low doses, and to 2.0 +/- 2.0 (p less than 0.001) under maximum doses. TNT intake decreased parallel also in a significant way. At the end of the trial, 37% of patients had become asymptomatic, and angina had reduced to less than two crisis a week in 33%. A clear relation doses-effect was observed. There was no alteration in laboratory exams neither in ECG. Seven patients had complications derived from the evolutional course of disease (2 IAM, 5 unstable angina and one sudden death). Adverse events were relatively frequent and the majority derived from vasodilator effect (tibial oedema 37%, flushing 17%, headache 23%).(ABSTRACT TRUNCATED AT 250 WORDS)