[Transdermal intoxication through broken opioid ampules : Accidental intoxication in a paramedic]. / Transdermale Intoxikation durch zerbrochene Opioidampullen : Akzidentelle Intoxikation bei einem Rettungsdienstmitarbeiter.

This article presents the case of an accidental transdermal opioid intoxication in a paramedic. During an ambulance flight mission for patient repatriation several ampules containing opioids were broken unnoticed inside the ampule kit in the outside pocket of the work trousers of the paramedic. He developed the typical clinical picture of opioid intoxication with clouding of consciousness, miosis, and respiratory depression. This necessitated continuous monitoring of vital signs as well as repetitive administration of naloxone under the improvised circumstances of a mission abroad.

Primary noncompaction of the ventricular myocardium from the morphogenetic standpoint.

This review compiles the current knowledge of normal and abnormal myocardial morphogenesis to facilitate an unambiguous diagnosis of primary myocardial noncompaction. During the early stages of development, the formation of trabeculae with the resulting increase in myocardial surface is a adaptation of the rapidly growing heart to improve nourishment by exchange diffusion from the cardiac lumen. Once the coronary vasculature has developed, the switch to cardiac nutrient supply through active circulation from the subepicardial space is paralleled by gradual compaction of the myocardial trabeculae. This results in a decrease of the inner, trabeculated myocardial layer with a parallel increase in thickness of the outer, compact myocardial layer. Similar to the direction of coronary arterial development, this process proceeds from the epicardium toward the endocardium and from the base of the heart to the apex. Based on developmental data, congenital myocardial noncompaction represents a failure of normal embryonic myocardial maturation. The time of arrest of this process will determine the extension of myocardial noncompaction within the ventricle. Whereas disturbances of myocardial microcirculation are frequent in these hearts, direct communications between the myocardial cavity and the coronary arteries (sinusoids) do not belong to this morphogenetic entity.

Systemic sarcoidosis after cardiac transplantation in a 9-year-old child.

Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.

[Infant botulism and sudden infant death syndrome]. / Säuglingsbotulismus und plötzlicher Kindstod: Eine kritische Bestandsaufnahme.

Infant botulism represents a distinct entity of botulism. Ingestion of the ubiquitously present spores of Clostridium botulinum leads to germination of the organism and neurotoxin production in the infant intestine. Symptoms typically develop gradually in contrast to classical food botulism in which an acute onset of symptoms shortly after the ingestion of preformed toxin in a food is characteristic. Microbiologically, the diagnosis is established by identification of Clostridium botulinum organism and toxin in stool specimen. However, positive results in these tests provide only indirect evidence for the clinical relevance of the neurotoxin since asymptomatic carriers have been found. The toxin irreversibly blocks the release of acetylcholin from the motoric end plate which results in muscle weakness and paralysis. Depending on the amount of toxin produced, infant botulism exhibits a broad clinical spectrum ranging from oligosymptomatic forms to a fulminant course with acute respiratory failure within hours leading to sudden death. Unrecognized mild forms or beginning muscle weakness can be a co-factor for other risk factors of sudden infant death (SIDS). In studies analyzing infants who died from SIDS, botulism bacteria or toxin were found in up to 20 % of cases. Infant botulism therefore represents an important differential diagnosis of unexplained and inconclusive muscular hypotonia in the first year of life.

Double-outlet right ventricle and overriding tricuspid valve reflect disturbances of looping, myocardialization, endocardial cushion differentiation, and apoptosis in TGF-beta(2)-knockout mice.

BACKGROUND: Transforming growth factor-beta(2) (TGF-beta(2)) is a member of a family of growth factors with the potential to modify multiple processes. Mice deficient in the TGF-beta(2) gene die around birth and show a variety of defects of different organs, including the heart. METHODS AND RESULTS: We studied the hearts of TGF-beta(2)-null mouse embryos from 11.5 to 18.5 days of gestation to analyze the types of defects and determine which processes of cardiac morphogenesis are affected by the absence of TGF-beta(2). Analysis of serial sections revealed malformations of the outflow tract (typically a double-outlet right ventricle) in 87.5%. There was 1 case of common arterial trunk. Abnormal thickening of the semilunar valves was seen in 4.2%. Associated malformations of the atrioventricular (AV) canal were found in 62.5% and were composed of perimembranous inlet ventricular septal defects (37.5%), AV valve thickening (33.3%), overriding tricuspid valve (25.0%), and complete AV septal defects (4.2%). Anomalies of the aorta and its branches were seen in 33.3%. Immunohistochemical staining showed failure of myocardialization of the mesenchyme of the atrial septum and the ventricular outflow tract as well as deficient valve differentiation. Morphometry documented this to be associated with absence of the normal decrease of total endocardial cushion volume in the older stages. Apoptosis in TGF-beta(2)-knockout mice was increased, although regional distribution was normal. CONCLUSIONS: TGF-beta(2)-knockout mice exhibited characteristic cardiovascular anomalies comparable to malformations seen in the human population.

Congenital polyvalvular disease: a review.

This review outlines the morphologic and pathogenetic characteristics of congenital polyvalvular disease. Two cases are used for exemplification. The macroscopic and histologic features of the valves as well as associated cardiac lesions and clinical syndromes are described, followed by a discussion of morphogenesis of this disease.

Mitral and aortic atresia associated with hypoplastic right lung, crossover segment of right lower lobe, and anomalous scimitar-like right pulmonary venous connection with inferior vena cava: clinical, angiocardiographic, and autopsy findings in a rare case.

A newborn female infant was found to have a unique and previously unreported group of anomalies: (1) mitral and aortic atresia with a highly obstructive atrial septum; (2) hypoplasia of the right lung with a crossover segment involving the right lower lobe; (3) normally connected pulmonary veins, two from the left lung and one from the right; and (4) a large anomalous branch of the right pulmonary vein of scimitar configuration that anastomosed with the normally connected right pulmonary vein and with the inferior vena cava (IVC). The scimitar vein appeared obstructed at its junction with the right pulmonary vein and at its junction with the inferior vena cava within the hepatic parenchyma. To our knowledge, this is the first report of a scimitar-like vein coexisting with mitral and aortic atresia and connecting both with the right pulmonary vein and with the inferior vena cava. The highly obstructed left atrium was partially decompressed by retrograde blood flow via the normally connected right pulmonary vein to the anomalous scimitar venous pathway and thence to the inferior vena cava via a pulmonary-to-IVC collateral vein.

The large window ductus: a surgical trap.

A rare window type of patent ductus arteriosus is reported that was large (15 mm in maximal transverse dimension) but had virtually no length and hence was externally invisible. The smaller aortic isthmus (4 mm in diameter), which was intrapericardial, was mistaken for the ductus and was inadvertently clip-occluded, leading to death. After a specific diagnosis is made, the large window ductus should be patched on cardiopulmonary bypass with a transpulmonary approach.

Pathologic anatomy of corrected transposition of the great arteries: medical and surgical implications.

BACKGROUND: Because the double-switch operation (atrial switch plus arterial switch) has recently become feasible in selected patients with congenitally physiologically corrected transposition of the great arteries, a detailed understanding of the pathologic anatomy is now mandatory for cardiologists, radiologists, and surgeons. METHODS: A detailed study of the pathologic anatomy, the clinical implications, and the surgical implications was undertaken on 33 postmortem cases with two ventricles. A companion study was also performed of 44 postmortem cases with functionally only one ventricle. Hence this was an investigation of 77 postmortem cases. RESULTS: Three main anatomic types of corrected transposition of the great arteries (TGA) with two ventricles were found: (1) TGA with solitus atria (S), L-loop ventricles (L), and L-TGA (L), that is, TGA [S,L,L] in 31 cases (94%); (2) TGA with solitus atria (S), L-loop ventricles (L), and D-TGA (D), that is, TGA [S,L,D] in 1 case (3%); and (3) TGA with inverted atria (I), D-loop ventricles (D), and D-TGA (D), that is, TGA [I,D,D] in 1 case (3%). Associated malformations resulted in 13 anatomic subtypes. In classical corrected TGA [S,L,L] with two ventricles, anomalies of the left-sided systemic tricuspid valve were present in 97%, with malformations of the left-sided systemic right ventricle in 91%. CONCLUSIONS: The findings in corrected TGA with two ventricles and in cases with single ventricle support the view that anatomic repair such as the double-switch procedure, or left-sided right ventricle bypass such as the modified Norwood procedure followed by the modified Fontan procedure, is indicated in selected patients.

Patch repair of subsemilunar conal septal defect resulting in severe left ventricular outflow tract obstruction.

A subsemilunar conal septal defect was closed at 8 months of age with a redundant Dacron patch that bowed into the left ventricular outflow tract, resulting in severe subaortic stenosis and massive left ventricular hypertrophy. Mistaken for cardiomyopathy or myocarditis, this rare complication of subsemilunar ventricular septal defect patch closure led to orthotopic cardiac transplantation followed by death.

Absent right superior vena cava in visceroatrial situs solitus.

Absence of the right superior vena cava (SVC) in visceroatrial situs solitus is rare (0.07% to 0.13% of congenital cardiovascular malformations), and little is known about the type and frequency of additional heart defects and arrhythmias. We reviewed previous publications and present 9 new cases. Based on 121 known cases, we found that this anomaly is typically characterized by: (1) persistence of the left SVC draining into the right atrium by way of the coronary sinus, and (2) left-sided azygos vein draining into the left SVC. Less constant features were: (3) additional cardiovascular malformations (46%), and (4) rhythm abnormalities (36%) that usually appeared related to the complications of old age. Since absence of the right SVC is clinically silent, its status should be assessed echocardiographically prior to invasive medical or surgical procedures. This is important to avoid various management difficulties during the following procedures: (1) implantation of a transvenous pacemaker, (2) placement of a pulmonary artery catheter for intraoperative or intensive care unit monitoring without fluoroscopy, (3) systemic venous cannulation for extracorporeal membrane oxygenation, (4) systemic venous cannulation for cardiopulmonary bypass, (5) partial or total cavopulmonary anastomoses; and (6) orthotopic heart transplantation and endomyocardial biopsies.

[Unilateral vs. bilateral lung transplantation in obstructive pulmonary disease]. / Uni- versus bilaterale Lungentransplantation bei obstruktiven Lungenerkrankungen.

BACKGROUND: The question whether patients suffering from end-stage emphysema who are candidates for lung transplantation should be treated with a single lung or with a double lung transplantation is still unanswered. METHODS: We reviewed 24 consecutive lung transplant procedures, comparing the results of 6 patients with an unilateral and 17 with a bilateral transplantation. PATIENTS AND RESULTS: After bilateral transplantation the patients showed a trend towards better blood gas exchange with shorter time on ventilator and intensive care compared patients after unilateral procedure. Three-year-actuarial survival was higher in the group after bilateral transplantation (83% versus 67%). There was a continuous improvement in pulmonary function in both groups during the first months after transplantation. Vital capacity and forced exspiratory ventilation therapies during the first second were significantly higher in the bilateral transplant group. CONCLUSION: Both unilateral and bilateral transplantation are feasible for patients with end-stage emphysema. Bilateral transplantation results in better pulmonary reserve capacity and faster rehabilitation.

Causes of death after the modified Norwood procedure: a study of 122 postmortem cases.

BACKGROUND: Although the results of the modified Norwood procedure as palliation for the hypoplastic left heart syndrome have improved considerably, in-hospital mortality remains high (28% to 46%). METHODS: To establish the causes of death and consider their therapeutic applications, we reviewed our pathology experience from 1980 to 1995, inclusive, regarding 122 patients who died after undergoing the Norwood procedure. RESULTS: The most important causes of death were found to be impairment of coronary perfusion (33 patients, 27%), excessive pulmonary blood flow (23 patients, 19%), obstruction of pulmonary arterial blood flow (21 patients, 17%), neoaortic obstruction (17 patients, 14%), right ventricular failure (16 patients, 13%), bleeding (9 patients, 7%), infection (6 patients, 5%), tricuspid or common atrioventricular valve dysfunction (6 patients, 5%), sudden death from presumed arrhythmias (6 patients, 5%), and necrotizing enterocolitis (3 patients, 3%). In 26 patients (21%), more than one factor appeared responsible for death. CONCLUSIONS: The leading causes of death after the Norwood procedure were found to be largely correctable surgical technical problems associated with perfusion of the lungs (36%), of the myocardium (27%), and of the systemic organs (14%).

Coronary artery bypass grafting after failed percutaneous angioplasty compared to direct coronary bypass grafting in patients with unstable angina.

With the increasing experience in percutaneous transluminal angioplasty (PTCA) for unstable angina surgical revascularization versus interventional angioplasty treatment has been controversial. From 1991 to 1993, 162 patients underwent coronary artery bypass grafting (CABG) for unstable angina. While 126 patients received primary surgery (group I), 36 had a PTCA first which was followed by emergency operation for complications (group II). In group II there were more cases of single-or double-vessel disease (p < 0.001) and none had left main stem stenosis (p < 0.0001), but there was no difference in ventricular function between the groups. The number of distal anastomoses, mean cross-clamp time, and usage of the internal mammary artery (IMA) were significantly lower in group II. The peri-operative mortality rate was comparable between groups (5.5% versus 8.3%; n.s.). Myocardial infarction occurred more frequently in patients after PTCA (8.3% versus 4.0%; p < 0.05) without increasing the rate of low output syndrome. In conclusion, emergency CABG after failed PTCA in patients with unstable angina carries an acceptable perioperative risk. Since the perioperative rate of myocardial infarction is higher and the IMA is used less frequently in this setting long-term results may be better with the direct surgical approach.