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The anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital coronary anomaly with a reported incidence of approximately 0.002%. Usually, the diagnosis is made by echocardiography leaving computed tomography or angiography only to diagnostic completion in doubtful cases or for interventional procedures.Herein we report a doubtful case of a patient with a diagnosis of coronary fistula between the right coronary and the pulmonary artery that proved to be an anomalous origin of the coronary artery from the pulmonary artery. The patient underwent corrective surgery with translocation of the coronary artery on the aorta.

Peripheral pulmonary artery stenosis is a quite rare condition that can lead to misdiagnosis, particularly in adults. We describe the case of a 48-year-old woman with multiple stenoses, challenging anatomy and Eisenmenger-like presentation, who was treated percutaneously with a coronary technique.

The ductus arteriosus is a physiological structure allowing shunt of blood from the pulmonary artery to the descending aorta during fetal life (unventilated lung). The incidence of isolated patent ductus arteriosus (PDA) in children born at term is about 1 per 2000 live births, accounting for 5-10% of all congenital heart defects. The pathophysiological significance and management of PDA vary according to the clinical context (isolated anomaly or as part of complex congenital heart disease), magnitude of the shunt, and stadium within the natural history. This review summarizes the embryogenetic, pathophysiological, clinical and therapeutic basic concepts for an updated approach to this particular heart disease commonly seen even by the cardiologist of the adult.

The aim of this work is to establish the accuracy of pulmonary magnetic resonance angiography (MRA) compared to multidetector computed tomography (MDCT) and pulmonary angiography in the diagnostic evaluation of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia. Eight consecutive patients underwent MRA, MDCT and pulmonary angiography. All patients were carriers of PAVMs amenable to endovascular treatment. The parameters we have evaluated were the presence, the number of fistulas and the diameter of the feeding artery of the malformation. In all cases, the findings of the three tests were comparable. In only one case, MR images were considered qualitatively poorer than MDCT, although sufficient for the recognition of the lesion located in the hilum. The diameters of the arteries showed no statistically differences between MRA and CT. In conclusion, the MRA technique was found to be valid and reliable in the diagnosis of PAVMs in patients with HHT.

Purpose: Evaluation of computed tomography (CT) pulmonary angiography parameters revealing pulmonary embolism (PE) severity with particular attention to pulmonary obstruction indexes. Comparison with clinical and hemodynamic data and determination of predictive role in the development of chronic pulmonary heart disease., Materials and Methods: This retrospective study analyzes 45 not consecutive patients from November 2007 to December 2008 with CT angiography diagnosis of acute PE. Included in the study are patients at the first documented episode of acute PE, with 6 month follow-up. Patients with severe pre-existent cardiopulmonary pathology or neoplastic diseases were excluded from the study. CT angiography evaluated right ventricular (RV)/left ventricular (LV) ratio, obstruction index according to Qanadli and Total Clot Burden (Ghanima score). PE indexes were compared with Troponin I measurement and echocardiography result; at last hospitalization and intensive care time were reported., Results: A significant association was found between Ghanima and Qanadli score: the two indexes are equivalent in quantification of pulmonary arterial obstruction (p<0.001). Among others CT parameters, the new Ghanima score evidenced the best accuracy to detect patients evolving to chronic pulmonary heart disease (76%). This value is higher than that of echocardiography (71%). Troponins showed highest accuracy (82%)., Conclusions: Ghanima score can be used in emergency CT angiography diagnosis as prognostic marker for a quickly risk stratification of pulmonary heart disease or death in patients with acute PE. This approach allows to obtain, with just one test, both the diagnosis and a rather accurate acute PE risk stratification.

A case of total anomalous pulmonary venous drainage (TAPVD) is reported, which according to Darling classification corresponded to infradiaphragmatic type (type III) and is associated to pulmonary arterial thrombosis and infarctions. It's emphasized the need of an accurate dissection of cardiovascular system in perinatal necropsies, specially in cases of sudden infant death. By an appropriate dissection, cardiovascular malformations can be recognized. Moreover, it's explained the pathogenesis of pulmonary arterial thrombosis and infarctions.

Tumors of the heart, while uncommon, represent an important chapter in cardiovascular disease. Potentially mortal, sometimes can be entirely removed by surgery, with complete recovery. Prognosis is related to histological features, localization, rapidity of growth, and quickness in diagnosing. They may be an occasional finding during chest X-ray, or two dimensional echocardiography. However, frequently they cause congestive heart failure or embolic phenomena. We report a case of voluminous angioma adherent to pulmonary artery, successfully removed by surgery. Cine computed tomography was decisive for accurate diagnosis, giving to the clinician useful information for surgical decisions.

The case refers to a term-fetus, small-for-date, with polydramnios, idiopathic hypereosinophilic syndrome and eosinophilic vasculitis of large, medium and small-size pulmonary arteries. The pulmonary arteritis was the only lesion observed in the fetus.

Lung specimens of 27 patients under six months of age with complete transposition of the great arteries (TGA), obtained at autopsy, were studied histologically. No evidence of hypertensive pulmonary vascular disease was found in patients under four months of age (23 patients), while obstructive intimal proliferations were seen in 2 patients, aged four and five months, with TGA and VSD. Since pulmonary vascular lesions in transposition with VSD appear early in infancy, timely palliative procedures, such as banding of the pulmonary artery, are strongly indicated when the radical corrective operation is to be postponed.

The case of a patient, died at 48 hours after the birth, with transposition of the great arteries and pulmonary vascular disease grade 3 is described. The evaluation of VPD was carried out in several sections from each lung, stained with H&E and elastico-Van Gieson. These histologic features is strongly in opposition with previous reports, in which pulmonary vessels exhibits only grade 1 or even grade 0 (hypoplasia of the media) modifications at the birth. However, many authors underline that children of same age and associated defects had widely differing degrees of VPD. Because our histologic findings, it is advisable an open lung biopsy and the evaluation of pulmonary vascular disease to plan the type of repair also early in life.

The authors report of a prenatal ultrasound diagnosis of hypoplastic left heart syndrome (HLHS) in a fetus at 36 weeks' gestation. The diagnosis-was substantiated by a postnatal two dimensional and M-mode echocardiogram and confirmed at postmortem examination. Pertinent findings were the morphology of ventricular cavities and of the mitral valve, and the abnormally increased ratio of right ventricular to left ventricular dimensions (approximately 2) as well as the pulmonary-to-aorta diameter ratio of 1,7. We stress the need of an accurate measurement of ventricular cavities to avoid a falsely positive diagnosis and the importance of a prenatal ultrasound diagnosis of congenital heart anomalies for a correct perinatal management.

We describe the cases of two neonates affected by the syndrome of pulmonary valve agenesis, in whom non-invasive diagnosis was possible by two dimensional echocardiography. The echocardiographic features we describe are the following: enlargement of the right ventricle in association with ventricular septal defect, malalignment type, dilatation of the right ventricular outflow tract, massive dilation of the main and branch pulmonary arteries, annular pulmonary stenosis. At the expected site of the pulmonary valve two ridges were seen, which did not have the typical motion of a fully developed valve. The ventricular septal motion was paradoxical in both cases. The diagnosis was confirmed at cardiac catheterization and the two neonates underwent operation unsuccessfully. The anatomic post-mortem examination confirmed the echocardiographic and angiographic findings. As successful treatment of this syndrome is difficult, we believe that a non-invasive echocardiographic diagnosis is very useful in order to avoid or, at least, delay a high risk cardiac catheterization.

The medical record and the lung sections were reviewed in 53 autoptic cases with pulmonary bone marrow embolism. In 31 cases the emboli could be ascribed to external cardiac massage, in one case to accident; in the other cases a definite aetiologic factor was lacking. The number of the emboli was largely variable from one section to another and sometimes very high (up to 16). The pulmonary bone marrow embolism has probably a clinical significance particularly when the underlying condition is severe.

The clinical, laboratory, haemodynamic, cardioangiographic and anatomo-pathological features are described in two patients in whom the association of "Primary Pulmonary Hypertension and Arterial Essential Systemic Hypertension" was noticed. On the basis of a hyperactive neurohormonal mechanism, a hypothetical, ethiological relationship between the pure hypertensive state of both the circulations is discussed. The occurrence of hypertensive systemic episodes and Raynard's phenomena lend weight to the hypothesis that neurohormonal mechanisms might participate in the pathophysiology of this syndrome.