Topography of cardiac ganglia in the adult human heart.

Published descriptions of the topography of cardiac ganglia in the human heart are limited and present conflicting results. This study was carried out to determine the distribution of cardiac ganglia in adult human hearts and to address these conflicts. Hearts obtained from autopsies and heart transplant procedures were sectioned, stained, and examined. Results indicate that the largest populations of cardiac ganglia are near the sinoatrial and atrioventricular nodes. Smaller collections of ganglia exist on the superior left atrial surface, the interatrial septum, and the atrial appendage-atrial junctions. Ganglia also exist at the base of the great vessels and the base of the ventricles. The right atrial free wall, atrial appendages, trunk of the great vessels, and most of the ventricular myocardium are devoid of cardiac ganglia. These findings suggest modifications to surgical procedures involving incisions through regions concentrated with ganglia to minimize arrhythmias and related complications. Repairs of septal defects, valvular procedures, and congenital reconstructions, such as the Senning and Fontan operations, involve incisions through areas densely populated with cardiac ganglia. The current standard procedure for orthotopic heart transplantation severs cardiac ganglia and their projections to nodal and muscular tissue. One modification of the current heart transplantation procedure, involving bicaval anastomosis, preserves atrial anatomy and the cardiac ganglia. Preservation of cardiac ganglia within the donor heart may provide additional neuronal substrate for intracardiac processing and targets for regenerating nerve fibers to the donor heart.

Pulmonary hypertension in polycythemia vera.

Thromboembolic events occur in about 27% of the patients with polycythemia vera and account for 31% of the deaths. These include cerebrovascular accidents, myocardial infarction, peripheral vascular occlusions, pulmonary infarctions, and venous thrombosis. We report two cases with polycythemia vera who presented with pulmonary hypertension in the absence of previous thromboembolic complications of any kind. One patient died suddenly, with evidence of extensive bilateral thrombosis of prelobular pulmonary arteries at autopsy. In the second patient, local thrombosis in the pulmonary vasculature or recurrent silent pulmonary emboli appear to be responsible for the development of pulmonary hypertension. After institution of anticoagulant therapy, he is able to maintain his functional status. The purpose of this report is to alert clinicians to the development of this insidious, but potentially fatal complication in patients with polycythemia vera.

Comparison of recombinant hirudin and heparin as an anticoagulant in a cardiopulmonary bypass model.

Recombinant (r) hirudin is a potent thrombin-specific inhibitor originally derived from the natural hirudin of the leech (Hirudo medicinalis). We have studied the efficacy of r-hirudin compared to heparin in a dog model of cardiopulmonary bypass (CPB) surgery. Two administration regimens were used for r-hirudin: Group I received 1.0 mg/kg intracardiac (i.c.) bolus then intravenous (i.v.) bolus at 30 min (n = 10); Group II received 1.0 mg/kg (i.c.) bolus with 1.25 +/- 0.04 mg/kg/h (i.v.) infusion (n = 8). Group III was given heparin 1.66 mg/kg (i.c.) bolus (n = 9). Aspiration of blood from the chest cavity revealed no significant difference between the three groups. Measurement of fibrin deposits in the pump line filter revealed higher amounts in the r-hirudin groups (P = 0.02). Decreases in platelets, fibrinogen and haematocrit due primarily to haemodilution were the same in each group. The bleeding time was less prolonged for r-hirudin than for heparin (p less than 0.001). No antagonist for r-hirudin was used; however, due to its short half-life, all coagulation parameters returned to baseline within 30 min after CPB. Since r-hirudin has no effect on platelets, is a poor immunogen, does not require a plasma cofactor, and may not require an antagonist, it may provide an alternative anticoagulant to heparin in CPB. Additional studies are, however, needed to optimize the dose and to evaluate other clinical aspects of r-hirudin.

Fungal endocarditis caused by Pseudallescheria (Petriellidium) boydii in an intravenous drug abuser.

We present a case of fungal endocarditis in a 42-year-old man with a history of intravenous drug abuse who required aortic valve replacement for severe aortic insufficiency. Cultures of the resected valve grew Pseudallescheria boydii. The patient subsequently developed persistent endocarditis of the prosthetic valve with systemic embolism. At autopsy, cultures of the prosthetic valve grew Monosporium apiospermum, an anamorph of Pseudallescheria boydii. Although fungal endocarditis is not uncommon in intravenous drug abusers, endocarditis caused by Pseudallescheria boydii is rare. It has been reported in only three other instances, and under circumstances that were different from ours.

Congenital laryngeal atresia.

Laryngeal atresia is a rare congenital anomaly requiring immediate tracheotomy as a lifesaving measure. A case of subglottic laryngeal atresia is reported and correlated with laryngeal embryogenesis. Three types of laryngeal atresia are described. Although tracheoesophageal fistula is commonly associated with laryngeal atresia, one must be careful not to confuse a pharyngotracheal duct with a tracheoesophageal fistula. A disproportionate number of reported TE fistulas associated with laryngeal atresia may result if an accurate distinction between pharyngotracheal duct and TE fistula is not made.

Parathyroid peliosis: report of a case and review of the literature.

Peliosis most commonly affects the liver and spleen and has been associated with exogenous and endogenous steroid hormones, particularly of the anabolic type. The finding of peliosis of the parathyroid glands, as well as of the liver, in an infant with adrenogenital syndrome indicates that other organs may be affected.

Fibrosing peritonitis in a drug abuser.

We report a case of fatal fibrosing peritonitis in a 28-year-old drug abuser. The extensive fibrosis, which was limited to the abdominal cavity, contained birefringent material. Silica was identified by spectrophotometric analysis. Silica is a component of talc (magnesium silicate) used as a drug filler. Relatively small amounts are known to induce fibrosis. The manner of introduction of this material into the peritoneal cavity is hypothetical and includes accidental direct peritoneal contamination during subcutaneous injections, a method used by drug abusers.

Rapid diagnosis of amniotic fluid embolism.

Acute respiratory failure developed in a 19-year-old primigravida 7 hours after undergoing cesarean section. The diagnosis of amniotic fluid embolism was established by viewing fragments of vernix caseosa in a pulmonary artery blood sample. Forty-eight hours after the cytologic diagnosis had been made, amniotic fluid material was no longer present in pulmonary arterial blood. The patient underwent supportive care in an intensive care setting and recovered completely.

Vitamin-D-dependent rickets type II. Resistance of target organs to 1,25-dihydroxyvitamin D.

Studies were done to determine the cause for hypocalcemia, secondary hyperparathyroidism, osteomalacia and osteitis fibrosa cystica in a 22-year-old black woman. The patient had normal serum 25-hydroxyvitamin D (14 ng per milliliter) and markedly elevated serum 1,25-dihydroxyvitamin D (137 pg per milliliter). Vitamin D3, 4000 units per day for four weeks, increased the serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D to as high as 29 and 297 pg per milliliter, respectively, and corrected the hypocalcemia and secondary hyperparathyroidism. The results suggest that the disorder results from impaired end-organ response to 1,25-dihydroxyvitamin D. We propose that the entity be called vitamin-D-dependent rickets Type II.

Adenoid cystic carcinoma of the vulva. Malignant cylindroma.

A case of adenoid cystic carcinoma (cylindroma) of the vulva is presented, reviewing the clinical course and response to therapy over a 5-year period. Only 9 previous cases had been reported by 1963. Several sources of cell origin had been previously suggested. Findings and histology in this case support a relation to a similar tumor occurring in other sites of the upper respiratory tract and salivary gland.

Anesthetic considerations in diffuse bleeding diathesis of uncertain origin.

This is a report of a case of a diffuse bleeding tendency in a pregnant woman who presented for emergency splenectomy with a tentative diagnosis of thrombotic thrombocytopenic purpura. The influence of multiple organ dysfunction in the selection of appropriate monitors and the anesthetic technic in such cases are complex.

Synovial sarcoma of the laryngopharynx.

Synovial sarcoma of the laryngopharynx is an extremely rare neoplasm. To date, only two such cases have been reported. We describe two additional patients in whom this lesion occurred as a primary neoplasm of the laryngopharynx. Hoarseness, upper respiratory distress, and dysphagia characterize the original complaints in laryngopharyngeal synovial sarcoma. The difficulties that may be encountered in histologic diagnosis are emphasized. Our findings suggest that an aggressive surgical approach is indicated. Adjuvant therapy with irradiation and the chemotherapeutic agent, doxorubicin (Adriamycin), may contribute to better survival rates.