Radioembolization with 32P-labelled glass microspheres for advanced hepatocellular carcinoma

We evaluated the efficacy and side effect of 32P-Labelled glass microspheres (32P-GMS) as a radioembolizer for patients with advanced hepatocellular carcinoma (HCC). 24 patients with unresectable HCC received internal radiation treatment of 32P-GMS. The tumor size varied from 3.6 to 18 cm. Hepatic arterial embolization was carried out through intraoperative or Seldinger's method. The mean absorbed radiation dose of the liver was 3250 rad (range from 1200 rad to 8000 rad). The radiation intensity within the tumor was 3.3 times stronger than in liver tissue. Not significant bone marrow renal toxicity was noted within 1 to 3 months. > 50% of tumor shrinkage was found in 17 cases, and < 50% of tumor reduction in 5 cases. The cumulative survival rate of 3, 6, 12, 18, 24 months was 92%, 75%, 54%, 33% and 29%. Hepatic arterial instillation of 32P-GMS appears to be safe and effective for unresectable HCC even with portal vein thrombosis.     

Identification of autolysosomes directly associated with proteolysis on the density gradients in isolated rat hepatocytes

BACKGROUND: More than 40% of patients who undergo curative resection of advanced colorectal carcinoma can be expected to have recurrence of the disease. The most frequent sites of recurrence are the liver (33% of patients) and lung (22%). Interest has therefore focused on treating hepatic or pulmonary metastases, or both, to improve the outcomes of these patients. Although surgical resection has become an increasingly accepted treatment for resectable localized hepatic or localized pulmonary metastases from colorectal carcinoma, the value of aggressive surgery for the removal of both hepatic and pulmonary metastases from patients with primary colorectal carcinoma remains to be clarified. METHODS: Data on 30 patients who had undergone resection of both hepatic and pulmonary metastases from colorectal carcinoma were included in the study. RESULTS: Independent, significant prognostic features were found to be the time that hepatic or pulmonary metastases occurred and the distribution of pulmonary metastases. Median survival times were 30 months (range, 7-108 months) after resection of both hepatic and pulmonary metastases and 48.5 months (range, 11-149 months) after excision of the primary colorectal tumor. Actuarial 1-, 3-, and 5-year survival after resection of both hepatic and pulmonary metastases was 86.7%, 49.3%, and 43.8%, respectively. No perioperative mortality occurred. There were three cases of minor morbidity, which the authors considered acceptable. CONCLUSIONS: Resection of both hepatic and pulmonary metastases from colorectal carcinoma may help to prolong the survival of a small group of patients with these metastases.     

An autopsy case of urinary bladder carcinoma with pulmonary infarction and subacute cor pulmonale caused by tumor embolization

Pulmonary tumor embolism is a common finding at autopsy but is difficult to diagnose clinically antemortem. We report an autopsy case of urinary bladder carcinoma associated with tumor emboli of the pulmonary arteries and subsequent pulmonary infarctions. An eighty-six-year-old man with bloody sputum showed multiple infiltrates on chest X-ray and multiple pleural based parenchymal lesions with truncated apex on computed tomography. The patient had a history of radiation therapy against urinary bladder carcinoma two years earlier. Transitional type carcinoma cells were identified from a urine sample obtained on admission. Three weeks later, the patient developed subacute cor pulmonale and died in severe respiratory distress. Postmortem examination revealed primary carcinoma of the urinary bladder. Multiple tumor emboli of pulmonary arteries and subsequent pulmonary infarctions were visible microscopically. There was a large amount of effusion in both the pleural and the abdominal space. The heart contained focal scarring and mild right ventricular hypertrophy and there was congestion of the lungs, liver, kidneys and spleen. Pulmonary tumor embolization may present at any stage of the patient's illness but rarely causes subsequent pulmonary infarctions. Cytologic examination of blood samples obtained from Swan-Ganz catheters may be useful in the diagnosis of tumor embolization.     

Analysis of cancer modality and distribution in China from year 1990 through 1992--an epidemiologic study

OBJECTIVE: Bronchopulmonary carcinosarcoma is a very rare tumor and the prognosis of patients with carcinosarcoma is assessed as unfavourable. The problems concerning diagnosis, therapy, and prognosis after resection treatment are discussed with reference to our seven cases and with consideration of the pertinent literature. METHODS: The retrospective data of seven patients with pulmonary carcinosarcoma were analysed. All were staged postoperatively according to the international TNM staging system. The diagnosis was verified by immunohistochemical investigation. The prognosis of the patients with carcinosarcoma was compared with the prognosis of patients with non-small cell carcinoma of the lung. RESULTS: Whether lung resection is the treatment of choice for these patients is of no relevance, because in most cases the preoperative diagnosis is incomplete, as only one component of the tumor, namely the epithelial one, is found in the biopsy specimen. The complete and correct diagnosis in five of the seven cases was not made before the resection had been performed and in the remaining two patients it was only made when tumor recurrence or metastases occurred. The prognosis of patients with carcinosarcoma of the lung is assessed to be comparable to that of patients with other pulmonary carcinoma: in this study survival times ranged from only 3 months (T2N3) to 4 years 6 months (T3N1). The causes of death of the patients with carcinosarcoma were local recurrence in four patients and metastases at distant sites in two. Two recurrent tumors as well as the metastases consisted only of the sarcoma component of the primary tumor histologically. CONCLUSION: One may suggest that the prognosis of carcinosarcoma might be determined by the sarcoma component of the tumor. Therefore the generally accepted therapies of soft tissue sarcomas should be adopted for the follow-up treatment of patients with pulmonary carcinosarcoma.     

Tetralogy of Fallot in adults

We present the clinical and hemodynamic profile of 33 patients, older than the 18 year with tetralogy of Fallot. Cardiac catheterization and selective angiocardiography were performed in all cases. We excluded patients with valvular pulmonary atresia associated with tetralogy of Fallot. Most of the patients were in functional class II or IV of the New York Heart Association and all presented with cyanosis. In the electrocardiogram of 23 patients we found right bundle branch block. None had significant cardiomegaly, 19 of 20 cases had reduced pulmonary blood flow and reticular pattern in the lung fields as observed in the chest X-ray. We did not find correlation between the age and the degree of cyanosis, but the level of arterial desaturation correlated with the functional class. The right ventricular systolic pressure was elevated in all patients. In all cases but one the pulmonary arterial systolic pressure were normal of slightly increased. One case with mild pulmonary infundibular stenosis had pulmonary systolic pressure similar to the systemic pressure. Multivariate analysis for functional class showed significant value for cyanosis. Systemic-pulmonary shunt was performed in 10 patients with hypoplastic pulmonary arteries plus reduced pulmonary blood flow, with one postoperative death. We did not find postoperative mortality in patients who underwent corrective surgery. The anatomic and functional behavior of patients who underwent surgery, allowed better tolerance to their heart defects.     

Pulmonary hypertrophic osteoarthropathy and clubbing of fingers in patients with lung cancer

We examined clinical characteristics of patients with primary lung cancer associated with clubbing of the fingers or pulmonary hypertrophic osteoarthropathy. Clubbing was observed in 12.5% of patients with lung cancer. Squamous cell carcinoma was frequently associated with clubbing. Clubbing was found in all clinical stages. PaO2 and PaCO2 were normal in patients with lung cancer, which suggests that neither hypoxemia nor hypercapnia caused the clubbing in these patients. Pulmonary hypertrophic osteoarthropathy was found in three patients with lung cancer (two men and one woman, mean age 49 years). The incidence was 2.9% among lung cancer patients with clubbing, 0.22% in all lung cancer patients, and was apparently lower than those in reports from outside Japan. One of these patients has stage IIIA squamous cell carcinoma, one had stage IV large cell carcinoma, and one had stage IV adenocarcinoma. In all cases bone scans were useful for diagnosis and for following the clinical course.     

Pulmonary infarcts can mimic pulmonary metastases from renal cancer

PURPOSE: Spontaneous regression of pulmonary metastases from renal cell carcinoma is a rare but well documented event. We present 2 recent cases that were radiographically consistent with pulmonary metastases from renal cell carcinoma but were pathologically shown to be pulmonary infarcts with no evidence of metastatic cells. Stable pulmonary infarcts can be misconstrued as metastatic disease in patients with renal cell carcinoma while resolving pulmonary infarcts may represent a subpopulation of patients with apparent spontaneous regression. Clinical implications of these findings are discussed. MATERIALS AND METHODS: Clinical and pathological data from 2 patients with large primary renal tumors, venous thrombi and lung masses were reviewed. Data from these cases, as well as pertinent urological and pathological literature, are presented. RESULTS: Although preoperative assessment was consistent with stage IV renal cell carcinoma, pathological examination of the lung masses in these patients showed no evidence of tumor cells. CONCLUSIONS: Pulmonary infarcts may mimic resolving or stable pulmonary metastasis in patients with renal cell carcinoma. Accurate clinical staging is crucial for the prognosis and treatment of renal cell carcinoma. Mistaking pulmonary infarcts for metastatic lesions can lead to inaccurate prognoses and inappropriate treatment.     

Arterial complications of pancreatitis: diagnostic and therapeutic aspects in 104 cases

PURPOSE: A retrospective study of 104 cases of arterial lesions related to pancreatitis was undertaken to examine the diagnostic and therapeutic benefits of arteriography compared with ultrasonography (US) and computed tomography (CT). PATIENTS AND METHODS: Data were acquired from responses to a questionnaire. Eighty-seven patients were men, 17 were women (age range, 21-80 years; mean, 48 years). These lesions were usually revealed by hemorrhage (70 cases), pain (69 cases), or both (46 cases). RESULTS: Arteriography was immediately positive in 90 of 93 patients in whom it was performed, but US and CT may also permit incidental discovery of silent lesions (17 cases). These lesions are often single (90%) and related to an arterial rupture in a pseudocyst (60%) or a pseudoaneurysm (48%). They involved the splenic (42%), the gastroduodenal (22%), and the small pancreatic arteries (25%). Of 32 cases in which embolization was performed, immediate success was achieved in all cases but bleeding recurred in 12 cases (37%). Treatment in 25 patients was a definitive success (78%), and five patients died of hemorrhage (16%). CONCLUSION: Arteriography remains essential for diagnosis of arterial lesions, and embolization may be indicated as a stabilizing preoperative procedure and also for immediately stopping hemorrhage.     

Clinical primary pulmonary hypertension: three pathologic types

Clinically, there is a group of patients with elevated pulmonary arterial pressure in whom the underlying cause is not apparent. The pulmonary arterial wedge pressure is not elevated. For such cases, the designation of primary pulmonary hypertension may be made clinically. From the clinical categorization of primary pulmonary hypertension, three distinct pathologic entities emerge, namely 1) plexogenic pulmonary arteriopathy, 2) recurrent pulmonary thromboembolism, and 3) pulmonary veno-occlusive disease. The plexogenic type is characterized initially by pulmonary arterial vasoconstriction with medial hypertrophy. Secondary proliferative intimal lesions, including the plexiform lesion, develop. Recurrent pulmonary thromboembolism is characterized by the presence of arterial thrombi of varying ages involving the microscopic-sized pulmonary arteries. Thrombi may be embolic in nature or may develop in situ. Pulmonary veno-occlusive disease is characterized by obstructive lesions of pulmonary veins and venules. The clinical presentation of the three pathologic types may be so similar that definitive diagnosis depends upon histologic examination of the lung from tissue obtained either by biopsy or at necropsy.     

Hepatic embolization through extrahepatic collateral pathways after hepatic arterial embolization for the hepatocellular carcinoma

Extrahepatic collateral pathways developing after repeated transcatheter arterial embolization (TAE) for hepatocellular carcinoma (HCC) make therapeutic arterial embolization for recurrent lesions extremely difficult. TAE was performed through the collateral pathways using a sophisticated micro-catheter with good trackability and pushability and a coaxial system. Twenty-three TAEs were undertaken through the collateral pathways in 13 patients with recurrent HCC who had extrahepatic collateral pathways after the previous hepatic arterial TAE. There were 69 extrahepatic collateral pathways, with partially obstructed hepatic arteries. On the average, three feeding arteries were seen in the liver. The main extrahepatic collateral pathways were the inferior phrenic artery and epicholedocal artery, 18 vessels and 29 vessels, respectively, accounting for about 80% of the total collateral pathways. TAEs were successful in all cases and the number of embolized vessels was 2.1 on average. The average time of the first collateral TAE after the initial conventional hepatic arterial TAE was 2.3 years. Excellent prognosis was observed with a one-year survival rate of 77% and 3-year survival rate of 38% after the collateral TAE. We consider that collateral TAE for recurrent HCC with obstruction of the hepatic artery is the procedure of choice, is technically feasible, and provides better prognosis for the patients.     

Surgical analysis for metastatic lung tumor from renal cell carcinoma

Metastatic lung tumor from renal cell carcinoma were studied in 29 cases. Eighteen patients were treated surgically, 11 were treated non-surgically. The overall 5-year survival rate with the patients of pulmonary resection was 53.5%, and that with those of conservative therapy was 0%, and this difference is statistically significant (p < 0.05). There was no significant difference in any characteristics such as sex, age, stage, grade, disease free interval, metastatic pattern and combination with or without interferon therapy. There was no significant difference in surgically treated patients with pulmonary metastasis in terms of any factors such as age, sex, stage, grade, disease free interval, pulmonary metastasis pattern, metastatic number, surgical procedure, combination with or without interferon therapy statistically. Analysis for the surgically treated patients with pulmonary metastasis from renal cell carcinoma shows no significant difference in prognosis with any characteristics. This result shows efficacy of surgery even if for the patients with synchronous bilateral multiple pulmonary metastasis from renal cell carcinoma.     

Hepatocellular carcinoma with portal vein tumor thrombus: embolization of arterioportal shunts

PURPOSE: To evaluate transcatheter arterial embolization in patients with hepatocellular carcinoma, portal vein tumor thrombus, and arterioportal shunts. MATERIALS AND METHODS: Ten patients with hepatocellular carcinoma, portal vein tumor thrombus, and severe arterioportal shunting were identified; in these patients, portal blood flow before embolization was hepatofugal. Embolization of arterioportal shunts was performed with steel coils that were introduced through a catheter during arteriography. After embolization, changes in portal hemodynamics and clinical signs and performance status of patients were evaluated; survival rates of patients with and patients without severe arterioportal shunting were compared. RESULTS: In all patients after embolization, angiography showed resolution of arterioportal shunting, and portography showed hepatopetal blood flow in the portal vein trunk. After embolization, performance status of five patients with initial scores of 2 or 3 improved. Ascites resolved in four patients and improved in four patients. One patient died of hepatic failure caused by rupture of esophageal varices 7 days after embolization. Median survival was 4.3 months, and the 6-month and 1-year survival rates were 45% and 12%, respectively. There were no significant differences between survival rates in patients with and patients without severe arterioportal shunting. CONCLUSION: Transcatheter arterial embolization of arterioportal shunts is a useful treatment for improving quality of life in patients with hepatocellular carcinoma.     

Arterial embolization of bone metastases: is it worthwhile?

Arterial embolization was performed in ten skeletal metastases occurring in nine patients: eight patients presented with renal cell carcinoma and one with bronchogenic carcinoma. Five metastatic lesions were located in the spine, one in the pelvis, three in the proximal humerus and one in the proximal femur. Selective arterial embolization was performed preoperatively in seven cases and as a palliative treatment in three cases. The embolic material used was polyvinyl alcohol particles, gelatin sponge and coils or a combination of these. Arterial embolization was technically successful in all patients achieving subtotal (> 90%) tumour devascularization in five metastases and a 75% devascularization in the remaining five lesions. In operative patients, median intra-operative volume of blood transfusion was 510 mL. Palliative embolization was followed by major pain relief in two cases and moderate relief in one case lasting from 7 to 26 months. Arterial embolization is an effective and safe adjunctive treatment of hypervascular bone metastases.     

Inferior vena cava filter used for unresectable renal cell carcinoma with tumor thrombi

A titanium Greenfield inferior vena cava filter was used for the treatment of 2 patients with unresectable renal cell carcinomas with tumor thrombi to prevent a fatal pulmonary embolism induced by tumor clots released during systemic interferon therapy and embolization of the primary tumor. After treatment, the size of the renal cell carcinomas at the primary site and the tumor thrombi decreased by 50%. There were no fatal pulmonary embolisms or complications related to the filter during the observation period (24 and 25 months) after therapy. This method may be useful in the prevention of a fatal pulmonary embolism induced by embolization and systemic interferon therapy in these patients.     

Pulmonary intravascular tumor emboli: dilated and beaded peripheral pulmonary arteries at CT

The diagnosis of pulmonary intravascular tumor emboli is difficult to establish both clinically and on conventional radiographic studies. Between 1985 and 1991, four cases of pulmonary intravascular metastases were demonstrated on computed tomographic (CT) scans from among 14,000 CT scans of the chest. A retrospective study of these cases was performed, including a review of chest radiographs. All four patients had invasive tumors, including an atrial myxoma, a renal cell carcinoma, an osteosarcoma, and a chondrosarcoma of the pelvis. Three cases had histopathologic documentation of pulmonary artery tumor emboli. At CT, all the patients demonstrated multifocal dilatation and beading of peripheral pulmonary arteries, primarily in a subsegmental distribution involving multiple lobes. Ossification of the pulmonary arteries occurred in one case of metastatic osteosarcoma. In two cases, small, peripheral wedge-shaped opacities distal to some abnormal pulmonary arteries suggested pulmonary infarcts. The finding of dilated and beaded peripheral pulmonary arteries at CT is highly suggestive of metastatic intravascular tumor emboli.     

Flow-cytometric evaluation of oxidative burst in phagocytic cells of children with cystic fibrosis

To assess the value of fine-needle aspiration (FNA) cytology for the diagnosis of amyloid, we retrospectively studied all FNA cases diagnosed as having amyloid during a 6-yr period (1990-1996). FNA was performed on both superficial and deep locations. A total of 6 cases containing amyloid was studied, including primary medullary thyroid carcinoma, metastatic medullary thyroid carcinoma to a vertebrae, multiple myeloma, squamous-cell carcinoma of the lung metastatic to a hilar lymph node, primary pulmonary amyloid, and amyloid tumor in a vertebral body in a patient with primary systemic amyloidosis. Despite the location or disease association, the cytologic appearance of amyloid in all cases was similar. On Diff-Quik stain, amyloid appeared as amorphous, irregular, waxy basophilic to metachromatic clumps of material. Papanicolaou stain revealed cyanophilic to organophilic clumps of material with occasional prominent fissures. In all 6 cases, amyloid was confirmed by Congo red stain and in 3 cases by a thioflavin T stain. In 4 of the 6 cases (67%), amyloid was associated with an underlying malignancy. In 3 cases malignant cells were admixed with the amyloid, and in another case malignancy was present at a distant site. We conclude that FNA biopsy is a helpful initial procedure for the evaluation of patients with amyloid deposits. The clinical implications of amyloid found in any particular body site include both benign and malignant conditions. The presence of an associated neoplasm must be especially considered in the differential diagnosis of amyloid deposits.     

How the left lung is perfused after ligating the left pulmonary artery in the pig at birth: clinical implications for the hypoperfused lung

The left pulmonary artery and ductus arteriosus were ligated in 14 pigs at birth. Animals were sacrificed at intervals from 2 to 24 weeks of age. In the right lung the pulmonary artery and in the left, either the distal pulmonary artery, bronchial arteries or both were injected. The fixed lung specimens were studied by arteriography, dissection and microscopic examination of serial and random sections of lung tissue. The bronchial arterial circulation to, and within the right lung appeared normal and was similar to that described in the human lung. In the left lung, the bronchial arterial circulation hypertrophied rapidly during the first 2 weeks, and large anastomoses between pulmonary and systemic circulations were found at the same sites as in the normal pig lung. The position and structural characteristics of the anastomosing arteries is described in the different types of broncho-pulmonary connection. In most animals aged 16 weeks or more, peripheral bronchial arteries immediately proximal to the anastomotic sites, developed intimal and medial proliferation. The left lung continued to grow although in all animals it was small. The axial pulmonary artery and its branches became smaller with age. These findings help explain how the lung is perfused and grows in children with congenital heart disease and an acquired collateral pulmonary arterial circulation.     

Carcinosarcomas of the female genital tract. A pathologic study of 29 metastatic tumors: further evidence for the dominant role of the epithelial component and the conversion theory of histogenesis

Carcinosarcomas of the female genital tract have generally been regarded as a type of sarcoma. Recent evidence suggests, however, that they may be more closely related to carcinoma. The histologic features of 29 carcinosarcomas with documented metastases were analyzed to study the relative importance of the carcinomatous and sarcomatous components and attempt to provide further evidence on the histogenesis of these neoplasms. Patients' ages ranged from 33 to 81 years (mean, 68). The primary tumor originated in the uterus in 17 cases, the ovary in 11, and the fallopian tube in one. Heterologous sarcoma was present in 21 of the primary tumors (72%). Myometrial invasion was present in all 15 of the uterine tumors treated with hysterectomy and consisted only of the carcinomatous component in 12 cases (80%). In two cases, which possibly developed as "collision"-type carcinosarcomas, the myometrium was separately invaded by carcinoma and sarcoma. Myoinvasive tumor consisted solely of sarcoma in one case. Lymphatic-vascular invasion was found in 10 of the primary tumors (eight uterine, two extrauterine) and consisted of pure carcinoma in all instances. The cellular composition of 62 metastases was evaluated. Of these, 51 metastases were diagnosed concurrently with the primary tumor in 21 patients (73%). Eleven metastases were diagnosed from 2 to 26 months after initial treatment. Carcinoma only was found in 43 metastases (70%), both carcinoma and sarcoma in 15 (24%), and sarcoma alone in four (6%). A total of 35 lymph node metastases occurred in 10 cases, consisting of carcinoma alone at 34 sites. The sole example of a purely sarcomatous lymph node metastasis occurred in one of the possible uterine "collision"-type tumors. Intraperitoneal metastases to serosal surfaces or the omentum occurred in 19 cases and consisted of both carcinoma and sarcoma in 14 and carcinoma only in five. Vaginal metastases occurred in four cases and consisted of only carcinoma in two, carcinoma and sarcoma in one and only sarcoma in one. Four patients had distant organ metastases, including one each to the liver (carcinoma only), breast (carcinoma only), bone marrow (sarcoma only), and brain (sarcoma only). Of the 51 concurrent metastases, only carcinoma was present in 37 (73%), both carcinoma and sarcoma in 13 (26%), and sarcoma alone in one. Of the 11 subsequent metastases, carcinoma alone was found in six (55%), sarcoma alone in three (27%), and both carcinoma and sarcoma in two (18%).(ABSTRACT TRUNCATED AT 400 WORDS)     

Utility of psychophysiological measurement in the diagnosis of posttraumatic stress disorder: results from a Department of Veterans Affairs Cooperative Study

OBJECTIVE: Predicting postrepair right ventricular/left ventricular pressure ratio has prognostic relevance for patients undergoing total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes during an intraoperative flow study. METHODS: Since January 1994, 15 consecutive patients (aged 64 +/- 54 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals were managed according to total neopulmonary arterial index. Seven patients with hypoplastic pulmonary arteries and a total neopulmonary arterial index less than 150 mm(2)/m(2) underwent palliative right ventricular outflow tract reconstruction followed by secondary 1-stage unifocalization and ventricular septal defect closure. The other 8 patients with a preoperative index of more than 150 mm(2)/m(2) underwent primary single-stage unifocalization and repair. The ventricular septal defect was closed in all cases (reopened in 1). In 9, such decision was based on an intraoperative flow study. RESULTS: Patients treated by right ventricular outflow tract reconstruction had a significant increase of pulmonary artery index (P=.006) within 22 +/- 6 months. Repair was successful in 14 cases (postrepair right ventricular/left ventricular pressure ratio = 0.47 +/- 0.1). One hospital death occurred as a result of pulmonary vascular obstructive disease, despite a reassuring intraoperative flow study. Accuracy of this test in predicting the postrepair mean pulmonary artery pressure was 89% (95% CI: 51%-99%). At follow-up (18 +/- 12 months), all patients are free of symptoms, requiring no medications. CONCLUSIONS: The integrated approach to total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals by preoperative calculation of total neopulmonary arterial index, right ventricular outflow tract reconstruction (when required), and intraoperative flow study may lead to optimal intermediate results.     

Scimitar syndrome in infancy

OBJECTIVES: The objectives of this study were to determine the anatomic and physiological factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome. BACKGROUND: Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made typically present with severe symptoms and have a poor prognosis. METHODS: The clinical, catheterization and imaging data of 13 consecutive infants with scimitar syndrome who underwent cardiac catheterization in the 1st 6 months of life were reviewed, with emphasis on the pulmonary artery pressure, pulmonary and cardiovascular anatomy, therapeutic interventions and outcome. RESULTS: Twelve of the 13 infants had pulmonary hypertension at the time of diagnosis. Six patients died despite specific treatment. Eleven of 13 infants had associated cardiac malformations and 9 had large systemic arterial collateral channels to the right lung. Seven patients had anomalies involving the left side of the heart, especially varying degrees of hypoplasia of the left heart or aorta, and six of these patients died. Ten patients underwent surgical or transcatheter therapy in the 1st year of life. Systemic arteries to the right lung were ligated in three patients and occluded by transcatheter embolization in four. Balloon angioplasty was carried out in two patients, one with stenosis of the left-sided pulmonary veins and one with stenosis of the anomalous right pulmonary vein. The latter had placement of a balloon-expandable stent. In both patients, pulmonary vein stenosis progressed. Six patients had surgical repair of associated cardiovascular anomalies, and two required repair of extracardiac congenital anomalies. Occlusion of the anomalous systemic arteries was generally associated with clinical improvement, but congestive heart failure and pulmonary hypertension recurred in those patients with associated cardiovascular anomalies, whose condition subsequently responded after correction of the shunt lesions. CONCLUSIONS: The severe symptoms and pulmonary hypertension found in infants with scimitar syndrome have many causes. Anomalous systemic arterial supply, pulmonary vein stenosis and associated cardiovascular anomalies play a significant role, and the ultimate outcome of individual infants depends on the feasibility of treating these anomalies in early infancy.