[Marfan syndrome and pregnancy]. / Marfans syndrom og graviditet.

Two cases of Marfan's syndrome with acute dissection in relation to labour are presented. One patient died of aortic rupture while the other was electively operated. The haemodynamic changes during pregnancy and labour can have catastrophic consequences. The Marfan patient must be well informed before pregnancy and carefully monitored during pregnancy and the peripartum period. Serial echocardiographic studies evaluating aortic root size and aortic valve function are essential. In case of severe cardiovascular manifestation the pregnancy should be interrupted.

[Perioperative risk in heart surgery. An original material and review of difficulties in correct comparisons with other materials]. / Perioperativ risiko ved hjerteoperationer. Et originalmateriale og en gennemgang af vanskelighederne ved korrekt sammenligning med andre materialer.

The purpose of this study was to present data concerning morbidity and mortality after cardiac surgery and to establish a method to make the presentation comparable to other reports. The main difficulty in comparing results of surgery of one institution with those of another is the lack of a simple and widely acceptable quantification of risk. A preoperative risk classification of patients requires readily available and objective data. The shortage of standardized criteria for comparing outcome was obvious as only a few comprehensive reports regarding preoperative predictors were found in the literature. The method of Tuman et al is based on 12 preoperative risk factors that are reasonably free of observer bias and practically obtainable. This method was used to report the results of 628 consecutive patients undergoing coronary revascularization or valvular surgery. Total in-hospital morbidity was 3.5% and mortality 1.0%. The most important predictors for postoperative morbidity were valvular surgery, advanced age, renal dysfunction, recent myocardial infarction and pulmonary hypertension. The system is most useful in predicting good outcome in low-risk patients. The identification of high-risk patients is valuable in spite of the limited predictive ability, by allowing special attention to be directed to the patient at risk.

Methaemoglobinaemia among neonates in a neonatal intensive care unit.

After detection of a few clinical cases of methaemoglobinaemia (methb) in our NICU, a prospective clinical study was undertaken to determine the extent of the problem and to identify the causes. Consequently, during the following 8 months all haemoglobin tests included simultaneous measurements of methb on an OSM 3 hemoximeter (Radiometer): 8% (n = 33) of 415 neonates were found to be methb positive (defined as > or = 6% methb). Mean methb was 19% (range 6.5-45.5%). Maximum methb concentrations were found on day 4-31 postpartum (mean 12 days) and the number of days with a positive methb sample ranged from 1 to 18 days (mean 6 days). About 40% of the neonates born at 25-30 weeks of gestation and 60% with a birth weight < 1000 g were methb positive. Also, there was a negative correlation between the size of the methb positive concentration and gestational age (r = -0.38, p = 0.02). Measurements of C-reactive protein and leucocytes, NADH reductase, pH, Cl, nitrate and nitrite were carried out in methb positive patients. The tests were repeated 1 week after cessation of methb. The only significant difference was an increase in NADH reductase at the second measurement. Likewise, a wide range of clinical parameters were registered and they occurred with a higher frequency among the methb positive patients when compared with a methb negative control group matched with regard to gestational age and the closest possible birth weight. The mean birth weight of methb positive patients was 1170 g and that of negative controls 1380 g (p < 0.006).(ABSTRACT TRUNCATED AT 250 WORDS)

[Aortic dissection--a life-threatening condition requiring immediate treatment]. / Aortadissektion--en livstruende tilstand der kroever hurtig behandling.

We present a retrospective examination of 70 consecutive patients (median age 55 years, range 20-71 years) operated for aortic dissection from January 1981 to October 1992. Four of these patients were operated twice, making a total of 74 operations. The operations were grouped according to the Stanford classification in Type A-acute (53%), type A-chronic (11%), type B-acute (26%) and type B-chronic (10%). Fifty-seven patients were males (81%) and 90% of the operations were performed as emergencies. Eleven percent of the patients had Marfan Syndrome. The peroperative mortality was 23% and the perioperative mortality was 39%. The 10 year survival including the perioperative mortality was 39%. The frequency of operative complications lasting for more than one month was 12% and some of these complications were caused rather by the aortic disease rather than the operation. Further improvement depends on the referral of these patients to cardio-thoracic surgical units on near suspicion of the condition for the earliest possible treatment. Furthermore, patients must be followed lifelong for recurrences in progression of chronic AD.

[Switching of the great vessels (arterial switch surgery) in transpositio vasorum]. / Omskifning af de store kar (arteriel switch operation) ved transpositio vasorum.

Since April 1992 the arterial switch operation (ASO) has been the treatment of neonates with transposition of the great arteries (TGA) at Rigshospitalet, Copenhagen. Thirteen mature neonates with TGA underwent ASO. Ten patients had simple TGA, two had TGA associated with a ventricular septal defect (VSD), and one had TGA with VSD and in addition moderate right ventricular hypoplasia. All patients survived the operation and are still alive. Perioperative bleeding was a problem in three cases. Eleven patients had an uncomplicated postoperative course. One patient had peri- and postoperative left ventricular failure and was reoperated after three months for a residual VSD. One child developed renal failure and needed peritoneal dialysis. The patients have been followed for 5.5 (range 0-12) months, they are all in good condition and thriving well. The presented early results after ASO justify continued recommendation of ASO as the operation of choice for TGA in neonates at Rigshospitalet.

[Surgical treatment of cardiovascular manifestations of Marfan syndrome]. / Kirurgisk behandling af kardiovaskulaere manifestationer ved Marfan's syndrom.

Thirteen patients with cardiovascular manifestations of Marfan's syndrome (MS) were admitted to the Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen, during an 11-year period (1981-92). One patient died preoperatively. Fifteen operations were performed in 12 patients. Four operations were elective, 11 were acute. Dilatation of the aorta (> 5 cm) accompanied by insufficiency of the aortic valve was the most frequent cardiovascular pathology, (13/13 (100%)). Aortic dissection was present in 8/13 (62%) patients. At the primary operation seven patients had the ascending aorta and the aortic valve replaced by a composite graft. Two of these also had the mitral valve replaced. Four patients had the aortic valve replaced and three patients underwent CABG (coronary artery bypass grafting) due to dissection into the coronary arteries. Three patients underwent a second operation. Two of these had composite grafts implanted, the last underwent CABG. The operative mortality at the primary operation was 17%, at re-operation it was 33%. This report illustrates the high risk of acute complications in patients with MS and emphasizes the importance of an early diagnosis of the syndrome, subsequent examination of the cardiovascular system and prophylactic elective surgery. Control of blood pressure and monitoring of aortic dilatation and valve function is recommended.

Composite graft replacement of the aortic valve and ascending aorta with Cabrol technique.

Replacement of the aortic valve, root and ascending aorta with the technique first described by Cabrol was performed on 17 patients. In five cases the aortic arch was also replaced. The indications were type A aortic dissection with aortic insufficiency (8 cases, with acute dissection in 7), native endocarditis with severe aortic insufficiency and aortic root abscess (3 cases), prosthetic endocarditis (3), and true aneurysm of the ascending aorta with aortic insufficiency (3). Acute surgery was performed in 15 cases (88%). The overall operative mortality was 41%. For the patients discharged from hospital the mean observation time was 30 months. None died, but in one case the right leg of the interposition graft became occluded and reoperation was required 40 months after the primary operation for acute type A dissection. The described technique of aortic valve and root displacement can be used in all cases in which use of a composite graft is indicated, except in situations where the coronary ostia and arteries are damaged by acute dissection.

Aneurysm of the ductus arteriosus in the neonate: three case reports with a review of the literature.

Ductus arteriosus aneurysm was demonstrated in three neonates by echocardiography. One case was already diagnosed in utero. One patient needed surgical resection, whereas in one case the aneurysm of the ductus arteriosus regressed spontaneously. In the third case the patient died of associated lesions. In a review of the literature, 61 previous cases of ductus aneurysm in infants less than 2 months were found. Ductus arteriosus aneurysm may be diagnosed by echocardiography both pre- and postnatally. A ductus aneurysm should be monitored closely by echocardiography. Since a high incidence of complications is reported, surgical resection is indicated if the aneurysm does not regress within a few days.

[Pulmonary arteriovenous malformations and fistulas]. / Pulmonale arteriovenøse misdannelser og fistler.

Pulmonary arteriovenous malformations are often associated with the Rendu-Osler-Weber-syndrome (40-65%) and involve high risks of serious complications, especially of cerebral character. Operative treatment minimizes morbidity and mortality, but presents problems in cases with multiple, bilateral lesions. Embolotherapy seems promising as an alternative to surgery in cases of widespread lesions. Based on the literature, active therapy is recommended to all patients with pulmonary arteriovenous malformations and fistulas. The only exception may be asymptomatic patients with small lesions who have no evidence of the Rendu-Osler-Weber-syndrome.

Large systemic collateral arteries developing late after total repair of tetralogy of Fallot.

A 22-year-old man with tetralogy of Fallot successfully underwent a corrective operation. Twenty years after total correction, large systemic collateral arteries (SCA) developed. The SCA arose from the right subclavian and internal mammary arteries. The SCA were not present at the time of total repair and thoracotomy had not been performed on this side. A closed-tube thoracostomy for postoperative pneumothorax may be responsible for the development of the large SCA.

Aneurysm of the ductus arteriosus. A review of the literature and the surgical implications.

One hundred and forty-four cases of aneurysms of the ductus arteriosus (DAA) have been reported in the literature of which 106 appeared spontaneously and 38 followed surgical treatment of a patent ductus arteriosus (PDA). Within the last few years there has been an increasing number of reported spontaneous DAA. However, the real incidence is presumably still underestimated. Aortography is a well established diagnostic method. In neonates, transthoracic echocardiography has shown convincing potential, whereas in older children and adults, transoesophageal echocardiography has yielded very promising results. Serious complications following spontaneous DAA are rupture, erosion, infection and thromboembolism. In infants younger than 2 months of age, the complication rate is 31%, in children between 2 months and 15 years, 66%, in adults, 47%. The rate of complications following postoperative DAA is even higher: 91% of the unoperated cases died due to rupture or infection. The operative mortality in children older than 2 months and adults is low. In the neonate group, 2 of 8 died during operation. The operative mortality in patients with postoperative DAA was 26%. Based on information from the literature, we suggest prompt surgical treatment of all spontaneous DAA in patients older than 2 months of age, and in all patients with postoperative DAA. In infants, a DAA should be closely followed with echocardiography, as spontaneous regression has been reported in this age group. If no regression is seen within a few days, it should be surgically corrected.

MR imaging of fat in and around the heart.

MR imaging is useful for characterizing collections of fat in and around the heart. This article illustrates the MR appearance of pericardial fat, epicardial and periaortic fat, intramural fatty involvement and intracavitary fat, with emphasis on the distinctions between fatty and nonfatty tumors.

Cardiac masses: assessment by MR imaging.

The purpose of this study was to assess the role of MR imaging for evaluating suspected cardiac tumors or paracardiac masses involving the heart. Sixty-one patients with clinical or radiologic evidence of cardiac masses were imaged with ECG-gated MR at 1.5 T (22 patients) or 0.15 T (39 patients). Fifty-one patients had echocardiography previously. Among the tissue diagnoses were myxoma (six); fibroma, rhabdomyoma, plasma cell granuloma, lipomatous hypertrophy of the atrial septum, mesothelioma, and thymoma (two each); and leiomyosarcoma, lymphoma, metastatic carcinoid, melanoma, malignant fibrous histiocytoma, hemangiopericytoma, and lung spindle cell sarcoma (one each). MR imaging demonstrated masses in 50 patients (82%); they were centered in the heart in 32, pericardial in nine, and juxtacardiac in nine. MR imaging provided diagnostic information that affected clinical management or surgical planning in 53 patients (87%), including 11 (18%) in whom cardiac mass was excluded by MR. The ability to provide a global view of cardiac anatomy and other unique capabilities of MR imaging give the procedure an important role in the diagnosis and preoperative assessment of cardiac masses.

Effect of folic and folinic acid on cytopenia occurring during co-trimoxazole treatment of Pneumocystis carinii pneumonia.

12 AIDS/ARC patients with or suspected of Pneumocystis carinii pneumonia were treated with co-trimoxazole and received supplementary folic or folinic acid to avoid peripheral blood cytopenia. Most patients developed decreased numbers of neutrophils and hemoglobin while receiving co-trimoxazole. Supplementary folate/folinate could not abolish the drug-induced cytopenia. Routine prescription of folinic acid is not recommended. Folic acid is cheap and may be beneficial and should be prescribed.