Idiopathic pulmonary arterial trunk aneurysm presenting as cor pulmonale: report of a case

An elderly woman who presented with cor pulmonale and intractable right heart failure was found at autopsy to have a thrombosed aneurysm of the pulmonary arterial trunk. This case is unusual in that no associated congenital cardiopulmonary anomaly, vascular myxoid degeneration, pulmonary parenchymal disease, or thromboembolism was found.     

Acute pulmonary thromboembolism after distal gastrectomy: an appraisal of the guidelines for preventing pulmonary thromboembolism/deep vein thrombosis

We report a case of acute pulmonary thromboembolism after gastrectomy. A 67-year-old woman was found to have gastric cancer and a giant lipoma in the ascending colon. We performed distal gastrectomy and enucleation of the ascending colon lipoma. On postoperative day 9, an acute pulmonary thromboembolism developed, and thrombolytic therapy was urgently performed. The 2004 Japanese guidelines for preventing pulmonary thromboembolism/deep vein thrombosis are discussed in relation to the present case.     

Thromboembolism of the general and pulmonary circulation (according to data from the Office of Pathological Anatomy of the Latvian Republic for 1976-1983)

Thromboembolism was found in 1060 (11.9%) of 8926 retrospectively studied autopsies. Thromboembolism of the lesser (pulmonary) circulation was the most frequent (7.3%), the domination of females was noted (62.8%). Peak in both males and females was within the age group of 70 to 79 years. The most frequent source of the pulmonary circulation thromboembolism was the lower limb veins. Thromboembolism of the pulmonary (31.9%) and greater (61%) circulation was most frequently observed in the ischemic heart disease. Monthly variations were not statistically significant.     

Interstitial deletion of the long arm of chromosome 6 associated with absent pulmonary valve

We report on a newborn infant with a deletion of 6q and absent pulmonary valve. His chromosome constitution was 46,XY,del (6) (q15q21). To our knowledge this is the first case with such combination. Some of his clinical features were found in common in 2 previous 6q- cases with the same breakpoints.     

Pulmonary tumor embolism to alveolar septal capillaries. An unusual cause of sudden cor pulmonale.

Although metastatic spread of tumor to the lungs is common, subsequent production of cor pulmonale is not. The involvement of pulmonary alveolar capillaries causing sudden cor pulmonale is very rare. We describe a patient who presented with chest pain and sudden shortness of breath. Autopsy disclosed diffuse pulmonary microembolism to septal capillaries caused by tumor cells from a squamous cell carcinoma of the cervix. To our knowledge, this is the second report of this kind of pulmonary tumor embolism.     

Fatal pulmonary arterial occlusive vascular disease following chemotherapy in a 9-month-old infant

Fatal pulmonary hypertension developed in an infant during the 7-month period in which he received, via a central venous catheter, combination chemotherapy for stage IV neuroblastoma as well as intermittent parenteral feeding. In a lung biopsy and at autopsy, small pulmonary arteries showed diffuse medial hypertrophy and peripheral muscularization, very extensive concentric intimal fibrosis, and focal eccentric fibrosis evolving from organizing thrombi. Pulmonary veins were normal. Hypothetically, chemotherapeutic drug therapy (possibly potentiated either by the parenteral nutrition or simply by the vehicular fluids causing volume loading of the pulmonary circulation) could cause occlusive pulmonary arterial disease by several mechanisms, but the association has not been described previously, although use of such drugs has been reported with pulmonary veno-occlusive disease.     

Spondylometaphyseal dysplasia, Sedaghatian type.

In 1980 Sedaghatian described an Iranian infant who died shortly after birth. At autopsy, he was found to have subacute myocarditis, cortical necrosis of kidneys, and adrenal and pulmonary hemorrhage. His skeletal abnormalities included mild rhizomelic shortness of his limbs and platyspondylyl and "laciness" of the iliac wings. In 1987 Optiz et al. described another Iranian infant with a similar perinatal course and roentgenograms. This infant was born to first cousins, suggesting an autosomal recessive single gene defect. We report our findings of another infant with a lethal course.     

Chronic hypoxia causes angiogenesis in addition to remodelling in the adult rat pulmonary circulation

Chronic hypoxia caused by migration of native sea-level dwellers to high altitude or chronic lung disease leads to the development of increased pulmonary vascular resistance and pulmonary hypertension. This altitude-induced hypertension offers no obvious benefit and may indeed be maladaptive. A major mechanism thought to contribute to the development of pulmonary hypertension is hypoxia-induced loss of small blood vessels, sometimes termed rarefaction or pruning. More recent evidence caused us to question this widely accepted concept including the potent angiogenic effect of chronic hypoxia in all other vascular beds and the demonstration that new vessels can form in the pulmonary circulation when stimulated by chronic infection and lung resection. We tested the hypothesis that chronic environmental hypoxia causes angiogenesis in the adult pulmonary circulation by using stereological techniques combined with confocal microscopy to examine the resultant changes in pulmonary vascular structure in rats. We found that chronic hypoxia resulted in increased total pulmonary vessel length, volume, endothelial surface area and number of endothelial cells in vivo . This is the first reported demonstration of hypoxia-induced angiogenesis in the mature pulmonary circulation, a structural adaptation that may have important beneficial consequences for gas exchange. These findings imply that we must revise the widely accepted paradigm that hypoxia-induced loss of small vessels is a key structural change contributing to the development of pulmonary hypertension in high altitude adaptation and chronic lung disease.     

A case of deep vein thrombosis and pulmonary thromboembolism after intravenous immunoglobulin therapy

Although high-dose intravenous immunoglobulin (IVIG) is generally considered a safe medication for various immune-mediated diseases, thrombotic events have been reported as a complication of the therapy. We report a case who developed thrombotic complications after receiving IVIG. A 56-yr-old woman with idiopathic thrombocytopenic purpura received IVIG at a dose of 400 mg/kg/day for five days. Three days after the administration of IVIG, the patient developed painful edema in the left leg. Lower extremity doppler ultrasound revealed deep vein thrombosis in the left leg. Chest computed tomography (CT) scan demonstrated a filling defect indicating thromboembolism of the right pulmonary artery. After three weeks of enoxaparin therapy, her symptoms and pulmonary embolism on CT improved. This case suggests clinicians should be cautious in the development of thromboembolism by administration of IVIG, especially in patients with thrombophilia.     

Plasticity in the development of handedness: evidence from normal development and early asymmetric brain injury

Previous research revealed that shifting patterns of hand preference in the first year of life are linked to infants' sensory-motor experiences as they learn to sit, creep, and walk. In this report, we examine whether new and different forms of locomotion and sensory-motor experiences similarly contribute to alter patterns of hand preference in early development. We examined the cases of three infants with unique developmental histories. Two infants adopted distinctive forms of locomotion in lieu of typical hands-and-knees crawling. One infant scooted using both hands and legs in a coupled fashion, while the other infant performed an asymmetrical, left-biased belly-crawl using only one arm to drag his body. The third infant suffered damage to his left-brain hemisphere shortly after birth and received intense physical therapy to his right arm as a result of it. We followed all three infants on a weekly basis and tracked changes in their reaching behavior, mode of locomotion, and postural achievements. The two infants with unique locomotor patterns displayed changes in hand preference that reciprocated the arm patterns that they used during locomotion. The infant who coupled his body for scooting began to reach bimanually, while the infant who adopted the left-biased belly-crawl developed a strong unimanual, right-hand, preference. The infant with left-hemisphere damage initially displayed a right-hand preference, then a temporary decline in preferred hand use as he began to cruise and walk, and ultimately resumed a right-hand preference in the 2nd year of life. This data is consistent with previous work showing that the development of hand preference in the 1st year of life is highly malleable and sensitive to a variety of new sensory-motor experiences.     

Pulmonary thromboembolism: frequency, site, and vascular change in 103 autopsy cases

For investigation of the incidence of pulmonary thromboembolism, the sites of thromboemboli, pulmonary vascular changes, detailed clinical and autopsy records, and histological sections of a series of 103 autopsied adults were reviewed. Pulmonary thromboembolism was observed in 38.8% of these cases. Pulmonary thromboembolism tended to be more frequent in older, younger, female, and cancer patients. Thromboembolism was found most frequently in arterioles, followed by muscular arteries. A significant correlation (P less than 0.02) was found between the incidence of pulmonary thromboembolism and the histological changes of endothelial protrusion and vacuolization of the intima in these small pulmonary arteries, but no correlation was found between that of pulmonary thromboembolism and constriction figures. These endothelial changes probably represent previous vasoconstriction. The present observations suggest a significant relationship between pulmonary thromboembolism and vasoconstriction of small pulmonary arteries and suggest that widespread vasoconstriction of pulmonary arteries is important in the pathogenesis and pathophysiology of this condition.     

Trousseau's syndrome in association with cholangiocarcinoma: positive tests for coagulation factors and anticardiolipin antibody

Thromboembolic events are reported to occur with a high frequency in the setting of malignancy. However, reports on an association between cholangiocarcinoma and pulmonary thromboembolism, thus far, are almost lacking. We present here an unusual case of a 56-yr-old patient presenting cholangiocarcinoma and unexplained pulmonary thromboembolism. The patient had been quite healthy before the diagnosis. Coagulation tests showed elevated levels of fibrinogen, fibrinogen degradation product (FDP), D-dimer, and IgM anticardiolipin antibody (aCL Ab). The thromboemboli were resolved 3 weeks after anticoagulant therapy using low molecular-weight-heparin. Then, follow-up coagulation tests showed a marked decrease to normal in aCL Ab titer as well as the normalization of FDP and D-dimer levels. In this case, we describe pulmonary thromboembolism caused by hypercoagulable state associated with cholangiocarcinoma and speculate that such a thrombotic phenomenon could be regressed by anticoagulant therapy.     

A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction

Pulmonary artery intimal sarcoma is a rare highly lethal disease, with additional retrograde extension to pulmonic valve and right ventricle being an extremely rare condition. It is frequently mistaken for pulmonary thromboembolism. We report a case of 64-year-old woman with progressive dyspnea initially suspected and treated for pulmonary thromboembolism. Her helical chest CT scan with 3 dimensional (3D) reconstruction combined with echocardiography revealed a compacting main pulmonary artery mass extending to the right ventricular outflow tract and the right pulmonary artery. After excision of the mass, the patient's condition improved dramatically, and the pathologic findings revealed pulmonary intimal sarcoma. This report emphasizes that helical chest CT with 3D reconstruction can be an important tool to differentiate the characteristics of pulmonary artery lesions, such as intimal sarcoma and thromboembolism.     

Post-ischemic rupture of the anterior papillary muscle of the right ventricle associated with persistent pulmonary hypertension of the newborn: a case report.

We report the case of a six-day-old male infant exposed in utero to a prostaglandin synthetase inhibitor, who presented pulmonary arterial hypertension, tricuspid insufficiency, and electrocardiographic signs of diffuse myocardial ischemia. The necropsy showed organizing infarction of the anterior and posterior right papillary muscles (probably occurred in utero) with complete rupture of the former, besides abnormal muscularization of the intraacinar pulmonary arterioles (persistent fetal circulation of the newborn). The authors suggest a possible relation between the myocardial ischemic and pulmonary hypertensive lesions since the prostaglandin synthetase inhibitor can induce precocious pulmonary arteriolar muscularization and constriction of the arterial duct, leading to right ventricular overload, thus facilitating the occurrence of papillary and subendocardial ischemia.     

Lethal skeletal dysplasia owing to double heterozygosity for achondroplasia and spondyloepiphyseal dysplasia congenita.

A male infant with lethal short limbed dwarfism is described. His father had spondyloepiphyseal dysplasia congenita and his mother had achondroplasia. It is believed that the infant inherited both of these disorders and that their combined effects resulted in early death owing primarily to severe pulmonary hypoplasia.     

Atypical pulmonary artery sling with diffuse-type pulmonary arteriovenous fistula

The case of a cyanotic infant with a rare combination of atypical pulmonary artery sling, imperforate anus, absence of the left kidney, interruption of the inferior vena cava, left side hemihypertrophy and diffuse-type pulmonary arteriovenous fistula is described. The clinical features were confusing, because of compounding abnormalities involving the respiratory tract and pulmonary circulation. The diagnostic approach to the etiology of cyanosis is discussed and the embryonic origin of pulmonary artery sling is reviewed.     

Pathological examinations of an enterovirus 71 infection: an autopsy case

We report an 8-month-old female infant with the fatal enterovirus 71 infection here. Clinically, she developed respiratory failure and severe pulmonary edema rapidly. Histologically, the lung specimen showed diffuse, severe pulmonary congestion and edema with focal intra-alveolar hemorrhage and typical features of acute encephalitis were easily identified under light microscope. Immunohistochemically, enterovirus 71 antigen was positive in the cerebella and brainstem. We measured the viral loads of different tissues and found that the brainstem and mesenteric lymph nodes showed the highest viral loads among all tissues. We hope that our case report may help to have a better understanding of the enterovirus 71 infection and provide clues to the prevention and treatment of this disease.     

Hypertensive Pulmonalarterienveränderungen bei chronisch-entzündlichen Lungenerkrankungen

Summary 150 cases of chronic inflammatory lung diseases of unknown aetiology and assumed hyperergic (immuno-reactive) pathogenesis were examined for hypertensive pulmonary arterial lesions and for chronic cor pulmonale. Hypertensive lesions of the small pulmonary arteries were found in more than half of the cases with chronic disorders of long duration, but were inconspicuous in diseases of acute progressive character. Hypertensive lesions were found regularly in chronic interstitial pneumonia, frequently in scleroderma and rheumatoid arthritis and occasionally in dermatomyositis and disseminated lupus erythematosus. Chronic Cor pulmonale occurred in 16% of the cases with hypertensive arterial lesions of grade I (hypertrophy of media) and in 50% of grade II/III (hypertrophy of media and intimai fibrosis). Interstitial lung fibrosis plays an important role in the pathogenesis of cor pulmonale: two thirds of the cases with interstitial lung fibrosis had developed cor pulmonale and all the cases with cor pulmonale also had interstitial lung fibrosis.Hypertensive arterial lesions of grade IV–VI according to Heath and Edwards (angiitis, plexogenic and angiomatoid lesions) have been described in severe cases of pulmonary hypertension (congenital cardiac shunts, primary pulmonary hypertension). In secondary forms of pulmonary hypertension, as represented by our material, these changes are of little importance.

     

Total anomalous origin of the coronary circulation from the right pulmonary artery

Origin of both coronary arteries from a branch of the pulmonary artery is rare and has not been reported as a cause of sudden unexpected death. We report autopsy findings of a 14-day male infant in previously good health who died suddenly. From the proximal right pulmonary artery arose a single coronary artery, which branched into the right and left main coronary arteries. The right proximal coronary artery coursed between the aorta and pulmonary trunk, and the left main traveled along the anterior proximal aorta. The distal course of the coronary circulation was normal. There was concentric subendocardial healing myocardial infarction. No other congenital anomalies were identified. Total anomalous origin of the coronary arteries from the pulmonary trunk or artery should be considered in cases of sudden unexpected death in infants.     

An autopsy case of ovarian hyperstimulation syndrome with massive pulmonary edema and pleural effusion

Ovarian hyperstimulation syndrome (OHSS) is the most serious complication of ovulation induction with exogenous gonadotropins, such as human menopausal gonadotropin and follicle-stimulating hormone. These hormones are considered to increase capillary permeability and cause third space fluid shift. We report an autopsy case of severe OHSS in a 28-year-old Japanese female. The patient developed bilateral chest pain and progressive dyspnea during the course of administration of human gonadotropins. Pleural effusion and hypouresis clinically disappeared 4 days after the onset of the symptoms, but the patient died suddenly of rapid respiratory insufficiency. Autopsy examination revealed massive pulmonary edema, intra-alveolar hemorrhage and pleural effusion without any evidence of pulmonary thromboembolism. Histopathological examination of the ovary demonstrated multiple well-developed follicle formations, consistent with OHSS. It is very important to recognize that massive pulmonary edema can occur in a patient with OHSS. To the best of our knowledge, this is the first autopsy report of a patient with severe OHSS.