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Tetralogy of Fallot is a congenital heart disease that accounts for 11-13% of the congenital cardiomyopathies. Stenosis and hyperplasia of the pulmonary arteries occur in a high proportion of them as this disease causes a native stenosis of the pulmonary branch, which can be surgically repaired with a stent. The use of balloon expandable intravascular stents is an alternative technique to further surgery in patients with congenital heart diseases. However, despite the gradual increase in their use, the limited number of procedures, combined with the wide anatomical variability and different characteristics of these patients, even in expert hands, stent implants are associated with a not inconsiderable incidence of complications. These are not always obvious and often require performing surgery in patients who have already had multiple interventions, thus increasing the complexity and the risk., (Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.)

We present the case of a 57-year-old male with left hilar squamous cell carcinoma infiltrating the pulmonary artery and in whom a sleeve bronchoplasty and angioplasty were performed using a bovine pericardial conduit. Three days post-operatively, graft thrombosis was detected; thrombectomy and graft reconstruction were performed with revascularisation of the graft., (Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.)

Behçet disease is a systemic disease with diverse clinical symptoms which vary according to the organs and systems involved. Typically, patients have oral and genital ulcers and episodic ocular involvement with periods of clinical improvement. We report an 18-year-old mole presenting with chest pain and hemoptysis and a history of ulcers in the oral cavity and scrotum. A chest CAT scan showed a mass located adjacent to the heart that a pulmonary arteriography defined as a left pulmonary artery aneurism. The patient was operated, excising left inferior pulmonary lobe. Due to skin and ocular involvement, the patient complied with criteria for Behçet disease. Immunosuppressive treatment with prednisone and azathioprine was started and the patient was discharged from the hospital.

Objective: We present our experience in using videothoracoscopy for the staging and assessment of resectability of lung cancer., Patients and Methods: Since 1993 we have carried out exploratory videothoracoscopy (EVT) for lung cancer staging and assessment of resectability. When intrapericardial vessel involvement is suspected, exploration by videopericardioscopy (VPC) is also useful for assessing resectability in these cT4 cases. Up to December 2007 we had studied 1381 patients with bronchogenic carcinoma. VPC was performed in 91 of these patients. In 45, the procedure was indicated because evidence of hilar and vascular invasion had been observed in the computed tomography or magnetic resonance images. In the remaining 46, it was performed as a result of EVT findings., Results: We were able to perform EVT in 1277 patients. In 104 cases this procedure could not be performed because of firm pleural adhesions. The tumor was resected after thoracotomy in 61 of these patients; thoracotomy was thus only exploratory in only 43 (3.1%). In 141 cases (10.2%) tumors were considered unresectable based on EVT, due to mediastinal invasion in 81 cases, pleural carcinomatosis in 38 cases, and both findings in 6 cases. Lobectomy was ruled out because of spread across a fissure or vascular invasion in 16 patients who were unable to tolerate pneumonectomy. In 61 of the 91 patients who underwent VPC we were able to perform lung resection; in the remaining 30, intrapericardial dissection was prevented by invasion of the pulmonary artery (17 cases), of the upper pulmonary artery and vein (6 cases), of the upper pulmonary artery and superior vena cava (2 cases), or of the left atrium and pulmonary veins (5 cases, in which the invasion was extensive)., Conclusions: EVT and VPC as a first step in lung cancer treatment require only a few minutes, do not contribute to morbidity, and avoid a significant proportion of exploratory thoracotomies.

The appearance of new and better magnetic resonance imaging (MRI) techniques have made the MRI a very important imaging method for the evaluation of thoracic vessels in pediatrics. The 3D angio-MRI using GD is capable of clearly demonstrating the morphology of the aorta and pulmonary vessels. The MRI may significantly reduce the number of angiographies needed and, in some patients, may even provide additional information to the angiography.

A case of Williams' syndrome in a 22 years old man, is described. Clinical data, as well as those of laboratory and of imageneology study, are reported. An electro-anatomical comparison permitted to verify the value of electrocardiographic signs of enlargement of the four heart chambers, due to a mixed overload. It permitted also to establish the value of the signs of the interatrial block, probably due to myocardial atrial fibrosis, and those suggesting hyperkalemia. The electrocardiogram always is very useful because it furnishes certain functional aspects permitting to allow structural inferences, in following subjects with congenital or acquired heart diseases.

Aortopulmonary fistulas secondary to thoracic aneurysms are difficult to diagnose and few cases are reported in the literature. We describe the case of a patient first admitted to another hospital with a diagnosis of right upper lobe pneumonia. After the patient had been a week in hospital without improving, a computed tomography scan of the chest revealed a saccular aneurysm of the aortic arch measuring 9 cm at its widest diameter. Hemodynamics and respiratory signs deteriorated a few hours after transfer to our hospital. Physical examination revealed a systolic murmur and poor peripheral perfusion. The patient was intubated and inotropic support was provided. Upon insertion of a Swan-Ganz catheter, given suspicion of septic shock, progressive elevation of mixed venous oxygen saturation was observed. Measurement of venous oxygen saturation was 74% in the right atrium and 93% in the pulmonary artery. Left-right shunt was diagnosed and an emergency aortography revealed the aortopulmonary fistula. Emergency surgery was performed with the patient in deep hypothermia and cardiocirculatory arrest. There were no adverse events, and postoperative recovery and clinical course were good.

Arterial aneurysm formation is a rare manifestation in Behçet's disease (BD). Aneurysm rupture is the main cause of death among BD patients. In recent years, endovascular treatment has been proposed as an effective and less invasive procedure compared with classical surgical treatment. We report the case of a 29-year-old man with BD who had two syncopes precipitated by pressure on a left cervical mass and hemoptysis. Cerebral magnetic resonance imaging (MRI) showed acute infarction of left basal ganglia. Other examinations demonstrated carotid and pulmonary aneurysms that were treated by endovascular methods. The patient received treatment with corticosteroids and immunosuppressive therapy. First of all, the pulmonary aneurysm was treated by means of coil embolization. After anticoagulant therapy was initiated, a covered stent was placed to exclude the carotid aneurysm at the level of extracranial internal carotid artery (ICA), with no immediate complications. After twenty-seven months of follow-up the patient remains asymptomatic. Endovascular treatment may be a definite therapeutic option for extracranial carotid aneurysms in BD, although longer term follow-up studies are needed.

Primary pulmonary hypertension (PPH) is a disease of un-known etiology that is characterized by an increase in pulmonary arterial pressure. Traditionally its treatment has been based on the administration of the vasodilator therapy with calcium channel blockers. At present there are some treatments which not only correct the vasoconstriction but also other pathogenic PPH associated effects, such a vascular remodelling and the thrombosis. These treatments include: a) analogs of prostacyclin with different administration routes; b) endothelin-receptor antagonists; and c) drugs that affect the metabolic pathway of nitric oxide. These pharmacological advances have reduced the number of pulmonary transplants in the patients with HPP, which is the last therapeutic alternative.

Congenital heart disease can increase or decrease pulmonary blood flow, pulmonary vascular resistance (PVR) or pulmonary artery pressure (PAP). PAP is the product of PVR and pulmonary minute volume (Qp), such that pulmonary hypertension (PHT) may develop as a result of an increase in either PVR or Qp or both. Given that the pulmonary vascular bed is a low pressure system with high flow, any increase in resistance would generate PHT. The normal value of PVR is 2 Woods units (mm Hg/l/min). Increased PAP is due to hypoxic lesions of the endothelium, which release proteolytic enzymes that alter the balance of metabolites of arachidonic acid, regulators of pulmonary vasomotor tone. Hypoxia and acidosis cause intense pulmonary vasoconstriction (hypoxic vasoconstrictor reflex). An increase of PVR is due to a combination of vasoconstrictive processes and remodeling, with hypertrophy of the pulmonary artery. Structural lesions are related to hypertrophy of the endothelium, the transformation of fibroblasts to myocytes and the decrease of the alveolar/arteriolar ratio with the formation of new vessels.PHT may be primary or secondary to another disease. Primary PHT is a rare genetic disease. The most common secondary forms of PHT in pediatrics are due to persistence of neonatal anatomy (neonatal PHT), to heart diseases with left-right shunt (CIV, DAP, etc.), to diseases of the pulmonary parenchyma (interstitial viral infection, mucoviscidosis), and complications of heart surgery. All congenital heart diseases can lead to PHT if not treated promptly. Clinical signs of PHT are highly non-specific: dyspnea, fatigue, syncopes, exercise intolerance, precordialgia, cyanosis and edema. The best approaches to diagnosis and prognosis are echocardiography and cardiac catheterization with vasodilators. Anesthetics that do not alter PVR should be used in such patients, who are sensitive to changes in pulmonary ventilation, to changes in cardiac output and to anesthetics. The treatment of PHT during intra and postoperative pediatric surgery is based on the use of high inspirated oxygen concentration (100%), an adequate sedation and the use of vasodilators (prostaglandin I2, nitric oxide, sodium nitroprusiate and milrinone).

The pulmonary artery aneurysm is a rare clinical entity that presents a low incidence and prevalence, of difficult diagnosis to be presented with poorly specific symptoms or also without symptoms, being detected in radiological studies as a widening or mediastinal mass. It can be uni or bilateral and presenting itself isolated or in the context of other sicknesses. The diagnosis of certainty is based in the realization of Echo-Doppler and other studies as a tomography or a magnetic resonance, the therapeutic option being so difficult, and according to cases, by an expectant or aggressive attitude.

Pulmonary artery intimal sarcomas tend to be presented with symptoms of pulmonary thromboembolism and grow regionally, with little capacity to metastasize. They probably originate from subendothelial cells, that become myofibroblasts. Knowledge of it is important to establish a presurgery diagnosis, with the possibility of a total resection, the only useful treatment until now. We report a case of a pulmonary artery primary sarcoma, in a 73 year old woman, admitted with hemoptysis and pleuritic chest pain, who died ten days after. Autopsy revealed an intraluminal mass at the pulmonary artery trunk, without regional nor distance involvement. Microscopic study showed a pleomorphic tumor with spindle and epithelioid cells, positive for actin, desmin and vimentin. All these data support the diagnosis of primary intimal sarcoma of the pulmonary artery. We want to emphasize the myogenic differentiation of the tumor, uncommon in previously reported cases.

We present two symptomatic patients of five and six months old with unilateral agenesis of main pulmonary branch (right and left) without associated anomalies. The diagnostic was made with magnetic resonance imaging and two-dimensional echocardiographic with Doppler color flow. We think that the magnetic resonance, must be used with priority in this pathology for being a non invasive diagnostic method, that is able to diagnostic the basic cardiopathy and the intracardiac and extracardiac associated anomalies. His association with the Doppler-echocardiography allow to ignore the arteriography upon to get a accurate diagnostic.

The hemodynamic and cardiovascular responses to a massive pulmonary embolism are: severe pulmonary hypertension, right ventricular failure and cardiogenic shock. The irreversible state of the latest condition and mortality could be due to a secondary right ventricle myocardial infarction, an entity which was first described in 1949. We report a necropsy case with massive pulmonary embolism and as a relevant finding a recent right ventricular myocardial infarction without significant obstructive coronary lesions. The relevance of right ventricle myocardial infarction as a major risk factor for mortality, its clinical and hemodynamic profile as well as the ischemic phenomena, are analyzed. It is emphasized also the importance of an early lysis of thrombus to rescue myocardium and to preserve right ventricle viability. This could be the first case reported in Mexico, in which the relationship between massive pulmonary embolism and right ventricle myocardial infarction is demonstrated as a determinant factor for mortality.

To demonstrate that the right ventricular infundibulum changes its spatial orientation in hearts with complete transposition of the great arteries, sixty five hearts with this malformation, with anterior and right sided aorta were studied morphometrically. The inlet apex and infundibular arises in the right ventricule were interrelated. The angles formed by the intersection of both arises were measured, and the results were compared with those obtained from a similar study in thirty five normal hearts. The angles obtained in the hearts with transposition of the great arteries were of 156.5 degrees, and those measured in the normal hearts were of 126.6 degrees. This difference was interpreted as an indicator of a vertical shift of the right ventricular infundibulum toward the right, to the same side in which the aorta is located and with which it is connected. In this way this outlet looses its leftward direction observed in normal hearts. It is concluded that although complete transpositions of the great arteries developmentally has its origin primarily in the truncus of the embryonic heart, the infundibulum is also involved modifying its position.

We present a symptomatic newborn infant with anomalous pulmonary artery (pulmonary artery sling) studied by magnetic resonance imaging. Of the ones diagnosed by this method, is the first case published in our country and the eight of all that have been reported until this moment. The different methods used for the diagnostic, are discussed. We conclude that, the magnetic resonance imaging is at the moment, the elective diagnostic method.

The goal of this paper has been to count and describe congenital heart disease associated with double conus. We studied 196 pathologic specimens from to the Pathologic Collection of the Department of Embryology of the National Institute of Cardiology "Ignacio Chávez" of México. We found 3% of double conus in classic tricuspid atresia with concordant ventriculoarterial connection, 53% with double outlet right ventricle, 16% with discordant ventriculoarterial connection, 91% with double outlet single ventricle, 8% with one outlet right ventricle with pulmonary atresia, 20% with one outlet left ventricle and 25% with one outlet single ventricle. The morphological data of the associated anomalies were gathered in an informatized sheet designed according the segmental and sequential approach to diagnose congenital heart disease. From our results we conclude that double conus cannot be considered pathognomonic of any congenital cardiopathy, although they appear associated more frequently with truncoconal anomalies and single ventricle. It is frequent the association between double conus and stenosis of one of them, specially the subpulmonary one. We found great association between left juxtaposition of the atrial appendages and double conus.

Six hearts specimens of cor triatriatum dextrum, eight with the Chiari's network, and 3 with a membranous remnant of the crista terminalis are studied. These anomalies are interpreted as varying degrees of persistence of the right valve of the sinus venous which reflect different stages of their morphogenesis. The most frequent congenital heart defects associated with these sinus remnants are the absence of a right atrioventricular connexion and pulmonary atresia with intact ventricular septum. There may be some haemodynamic factors in the right atrium such as partial of complete obstruction between the atrium and the right ventricle which explain the fact that the valve is not reabsorbed. In our specimens the persistent right valve divided the right atrium into two compartments; one is located in the medial sinusal portion where both cavae veins and coronary sinus are connected; the other is lateral compartment which represents the primitive right atrium. It must be noted that the persistent right valve favors blood flow to the left atrium through the patent foramen ovale or through an atrial septal defect deviating the course of the blood away from the right ventricle. This could cause the hypoplasia of the right ventricle. When the valve bulges it obstructs the blood flowing into the right ventricle. The diagnosis of these malformations can be made by echocardiographic, angiocardiographic procedures and nuclear magnetic resonance.

The present report deals with the clinical history and autopsy findings of a 41 year-old male patient with hepatic cirrhosis, surgical splenorenal shunt and severe pulmonary hypertension. Since the original description of Mantz and Craige several series have been reported, both in children and in adults, of the association between pulmonary hypertension and liver disease and/or portal hypertension. However, the type of hepatic alteration associated to pulmonary hypertension and the mechanisms responsible for this association remain unclarified. In the present case prominent plexiform and/or angiomatoid lesions of the pulmonary vasculature were observed, prompting the authors to speculate on the possibility that non-identified vasoconstrictor substances might contribute to the production of morphological lesions in the arterio-venous pulmonary shunts.

Connective tissue diseases may be associated with both an obstructive or a restrictive ventilatory defect. RAdiographic and microscopic findings usually reveal interstitial disease. This study describes a series of 11 patients with a connective tissue disease. Radiologically an interstitial pattern was observed, however, lung biopsies disclosed a bronchiolar lesion in which no pathology could be detected in alveolar septa. Vascular lesions were present in some cases an correlated roughly with time of onset. The bronchiolar lesions had no correlation with time of onset. We conclude that "interstitial disease" diagnosed by X-rays and pulmonary function studies may in fact, be caused by thickening of bronchioles and some blood vessels.

The present report is based on the study of 28 cases of pulmonary atresia with intact ventricular septum, all of them necropsically and/or angiographically verified. A microscopic study of the wall of both ventricles was performed in the 20 necropsy cases. The size of the right ventricular cavity, a fact of great surgical significance, did not correlate with the electrocardiogram and varied from diminute to very large. In two cases necropsically proved there was as associated atresia of the infundibular, in one of them existing additionally an Ebstein malformation of the tricuspid valve, which could be angiocardiographically diagnosed. The surgical mortality, in our hands, is 64.3%. All survivors have a small right ventricle.

The relationship of the great arteries with their respective ventricles in the three toncoconal morphologies depends on the orientation and rotation of the septum of the trunk and cone. In this work we study a case of crossed great arteries and another of partial distortion of the great arteries and one of transposition of the great arteries, all with arterioventricular concordance. In the anatomic and angiocardiographic examples an analysis is made of the arterioventricular relation in each type of arterial pedicle. We say that arterioventricular concordance exists with crossed great arteries when the pulmonary originated from the anterior infundibulum and its direction of right to left or from left to right in the frontal plane shows the spacial position of the anatomically right ventricle. In the transposition of the great arteries or in the partial distortion of the great arteries with arterioventricular concordance the aorta in the frontal plane shows the spacial position of the anatomically right ventricle. In the first group the aorta originates from the anterior infundibulum while in the second, from the posterior infundibulum. We review the examples of arterioventricular discordance diagnosed by necropsy or by laboratory studies. We study 5 cases; 1 with crossed great arteries, 3 with transposition of great arteries and the last with partial distortion of the great arteries. In the arterioventricular discordances with crossed great arteries in the lateral position, we observe that the pulmonary artery originates from the anterior infudnibulum whereas in the transposition, the aorta emerges from the anterior infundibulum. In the partial distortion of the great arteries the vessels are side by side or the aorta a little bit anterior to the pulmonary which is connected with the anterior infundibulum. In the posteroanterior incidence when there exists an arterioventricular discordance with crossed great arteries, the direction of the pulmonary does not indicate the spacial position of the anatomically right ventricle. In the same way, in the transposition and in the partial distortion of the great arteries the aorta does not indicate the spacial position of the anatomically right ventricle. On the basis of the evidence obtained by experimental and descriptive embriology, the morphogenetic processes responsible for the arterioventricular relations are presented. The anatomic specimens of each example is analyzed and the adequate parameter for the diagnosis of the distinct varieties of arterioventricular relations are presented. Finally a review is made of the few cases found in the literature and the differential diagsis between the concordant and discordant troncoconal malformation is established.

This paper manifests the importance of the systemic-pulmonary collateral circulation in congenital heart diseases. It shows that through the angiographic and hemodynamic study you should recognize the number, origin, course, and size of each of these arteries because during the corrective surgical treatment of certain types of heart diseases, these arteries should be tied off to prevent cardiac overload during the procedure and to prevent intrapulmonary short circuits from left to right in the postoperative period. The knowledge of the central and peripheral pulmonary arteries is important and because of this it should be remembered that the area of the section of the pulmonary arteries, right to left, together should be at least the equivalent of half of the area of the section of the aorta in its diaphragmatic level, the diameter which permits an acceptable cardiac output after the intervention. If the arteries are adequate a plan of surgical intervention which permits the control of the collateral systemic-pulmonary great arteries should be drawn up.

Report of a post-mortem study in 34 infants having presented pulmonary hypertension. Ages were between 16 days and three years old. One of the patients showed pulmonary lesion because of plexogenic arteriopathy of unknown etiology. The other 33 cases had congenital heart malformations. Macroscopic data as heart weight, thickness of the wall of the right ventricle and circumference of the pulmonary artery trunk were analized. Lesions developed in the pulmonary artery and its branches as well as those of the pulmonary veins are described. Different patterns of the hypertensive vascular disease are refered, emphasizing their evolution with the age of the patients. Some of the cases are particularly analized because of clinical, physiopathological or morphological peculiarities.

Ten cases with pumonary atresia with intact interventricular septum were studied; they were divided in type I (6 cases) and type II (4 cases). In the cases with necropsy no distinct characteristics were observed in comparison to those reported in the literature. Cyanosis, heart failure, cardiomegaly and pulmonary oligohaemia were present in all cases. No correlation was found neither between right atrial middle pressure and the P wave amplitude (r = 0.014), nor between the AQRS and the right ventricular systolic pressure (r = 0.039). All the cases from type II showed hypertrophy of the two ventricles and only one case from type I showed right ventricular hypertrophy. In nine cases right ventricular hypertension was registered. Right atrial middle pressure and right ventricular telediastolic pressure were found increased in nine cases; the other one showed normal values (type II). The angiocardiographic study is a necessary diagnostic procedure. By means of it is possible to determine the ventricular cavity size, the right ventricular infundibulum characteristics and the atresic segment size.

We studied 15 patients with the diagnosis of tetralogy of Fallot by means of conventional and axial angiocardiography. Axial studies were performed in the four chamber view (vertical and horizontal X-ray beams) and in the sitting-up view (vertical X-ray beam). With the right ventriculogram we analyzed the anatomic characteristics of this chamber, infundibular stenosis and the pulmonary branches. Left ventriculograms were performed in order to rule out other less common associated defects. In the sitting-up view we analyzed the characteristics of the right ventricular infundibulum and the pulmonary valve, artery and branches. None of our patients underwent aortography or coronary artery angiography. We conclude that utilization of axial angiocardiography is useful in tetralogy of Fallot because it permits a better appreciation of the anatomy of this malformation, the presence or absence of stenosis of the pulmonary branches and other less frequent associated defects. Aortography or coronary artery angiography must be performed in children in which the anatomy of the coronary arteries cannot be determined by either left or right ventriculograms.

The present study comprises a pathological review of 18 necropsy cases with nonspecific aorto-arteritis (Takayasu's disease), autopsied at the Instituto Nacional de Cardiología of Mexico up to 1976. There were 11 females and 7 males with an average age at the time of death of 21.5 years. A comparison according to the distribution and extention of arterial lesions disclosed that our cases are often less classical than those described from Japan. The principal arterial changes were stenosis, occlusion, dilatation and aneurysm formation in the aorta, its branches and main pulmonary artery. More often than not the aorta was extensively involved. The aortic arch syndrome and renovascular hypertension were almost equally present in this serie and an unusual high incidence of coronary artery involvement (33%) leading to myocardial infarction (50%) also was observed. The significance of the intrapulmonary vascular changes in Takayasu's arteritis is discussed.

We report the first case in the Spanish literature of pulmonary hypertension (PHT) caused by vascular sclerosis in a parenteral drug abuser female. The clinical diagnosis of PHT was confirmed in the necropsy study, which ruled out granulomas and discovered only vascular sclerosis and hypertrophy as causative factors of the clinical picture. The different etiological and pathogenetical mechanisms reported in the literature as causing PHT in this type of patients are reviewed.

Two cases of double outlet of the right ventricle with an anterior aorta to the left of the pulmonary artery are described. Both in situs solitus. One had an anterior interventricular communication, and other had a middle posterior communication. The importance of the aortic component of the second sound is analyzed with regard to its localization and intensity. An analysis is also made of the morphology of the cardiac silhouette and the peculiarities of the electrocardiogram as diagnostic aides in the malformation. Considerations are made of the distinct theories which explain the malformation. It is clearly shown that a correct diagnosis is necessary since this cardiopathy may be treated surgically.