Single ventricle. New embryological-anatomical classification. I. Pathology and physiology

The different nomenclatures which have been given to this malformation are discussed, defining the single ventrical as a single ventricular chamber without an interventricular septum and generally with two well defined atrioventricular orfices, although in some cases there is only one mitral-tricuspid ring. Twelve cases of single ventricle, proven by necropsic study, were examined. A new embriological-anatomical classification was proposed based on the location of the outflow tract (concordant or discordant in relation to the situs viscerae of which it is a part) and the troncoconal morphology (crossed great vessels, transposition of the great arteries, and truncus arteriosus), presenting some examples of these malformations. The pathological characteristics of the malformation in each of the established groups is described, as well as the physiopathology in the most frequent varieties. The defects associated with single ventricle are studied.     

Nocardia abscess of the choroid plexus: clinical and pathological case report

OBJECTIVE: Cerebral Nocardia abscesses are rare, accounting for approximately 1 to 2% of all cerebral abscesses. Prompt aggressive surgical treatment involving craniotomy and excision of these lesions has been advocated by many authors, because these lesions have significantly higher morbidity and mortality rates than do most other cerebral abscesses. We report an atypical presentation of cerebral nocardiosis localized to the choroid plexus of the lateral ventricle. CLINICAL PRESENTATION: A 56-year-old man presented with a 3-week history of fever, cough, and progressive headache and an ensuing 3-day history of progressive lethargy, confusion, and gait ataxia. Radiographic studies demonstrated a loculated contrast-enhancing left lateral ventricular lesion with significant perilesional parenchymal edema that was thought preoperatively to be a neoplasm. INTERVENTION: The patient underwent a craniotomy for resection of the lesion. Intraoperatively, a reddish gray lesion with purulent exudate was encountered within the left lateral ventricle intimately adherent to the choroid plexus as well as to the ependyma and subependymal veins. A frozen section demonstrated an organizing abscess wall. The lesion was resected in its entirety, and multiple cultures were sent for analysis. CONCLUSION: Microbiology cultures grew Nocardia asteroides. A course of intravenous antibiotics was started, which included trimethoprim-sulfamethoxazole, amikacin, and ceftriaxone. Two weeks after surgery, at the time of discharge, the patient's neurological status had improved considerably. Although Nocardia abscesses have been documented to occur throughout the central nervous system, the presentation of a lesion confined to the choroid plexus of the lateral ventricle with significant parenchymal edema is unusual and demonstrates that Nocardia abscesses must be considered in the differential diagnosis of a contrast-enhancing intraventricular mass lesion involving the choroid plexus.     

A rapid, specific membrane filtration procedure for enumeration of enterococci in recreational water

BACKGROUND: Perioperative and long-term problems associated with the Fontan circulation are substantial. There has been an exploration of extending the limits of a biventricular ventricular repair by using a superior vena cava-to-pulmonary artery anastomosis. This type of repair is known as a 1 1/2 ventricle repair. METHODS: Patients having defects of the pulmonary ventricle in size or function have undergone 1 1/2 ventricle repairs with or without creation of an atrial septal defect. Repairs with tricuspid z values as small as -10 and predicted pulmonary ventricular volumes as low as 30% have been reported. The 1 1/2 ventricle repair technique has also been used in special situations associated with an arterial switch or double switch procedure. RESULTS: Mortality has ranged from 0% to 12%. Complications have included persistent elevation of superior vena cava pressure, intermittent periorbital edema, and 1 superior vena caval aneurysm requiring takedown. There appears to be an increased risk of perioperative pleural effusions and chylothorax. Protein-losing enteropathy and chronic atrial arrhythmias have not been present. CONCLUSIONS: Successful 1 1/2 ventricle repairs have been reported for morphologically small or poorly functioning pulmonary ventricles and special situations. Intermediate-term follow-up is favorable when compared with reported outcomes for the Fontan circulation.     

Function of the residual myocardium after infarct and prognostic significance

Changes of the left ventricle after myocardial infarction are characterized by geometric, structural, and vascular alterations, which have been summarized under the term "remodeling". This process takes place in the infarct region as well as in the surviving myocardium. Depending on to the size of infarction and the degree of neurohumoral activation, the left ventricle demonstrates diastolic dysfunction which may finally lead to systolic failure. The residual myocardium develops progressive myocyte hypertrophy and interstitial fibrosis. These structural alterations are due to changes in loading conditions and stimulation of the neurohumoral system with an activation of local paracrine and autocrine factors. Myocardial function can be assessed by different non-invasive (echocardiography, radionuclide ventriculography, magnetic resonance imaging, etc.) or invasive methods (e.g., simultaneous pressure-volume measurements). "Myocardial tagging" based on magnetic resonance imaging allows the assessment of 3D-motion of the left ventricle by labelling specific myocardial regions with a rectangular grid. A systolic "wringing" motion with clock-wise rotation at the base and counter-clockwise rotation at the apex has been described in normal subjects. In the ischemic myocardium, delayed relaxation with a prolonged back-rotation (untwisting) has been reported during early diastole, whereas decreased systolic contraction with delayed diastolic rotation has been observed in non-Q-wave infarction. In patients with anterolateral aneurysms, a complete loss of systolic rotation has been demonstrated. The prognostic significance of LV "remodeling" has been emphasized by several authors: The size of infarction, LV volume, LV ejection fraction, as well as the degree of neurohumoral activation have been identified as being associated with an unfavorable clinical outcome. Yearly mortality rates have been reported to range between 15 and 17% in patients with large infarcts and marked LV dilatation and between 3 and 7% in patients with small to medium-sized infarcts.     

Surgically resected brain stem cavernous angioma in an infant

An infantile case of surgically resected brain stem cavernous angioma is presented. This 21-month-old boy was admitted to our department after repeated severe pontine hemorrhage. A deeplying lesion with hematoma was successfully removed via the IV ventricle approach. Few infantile cases of this lesion have been presented previously, and the approaches to brain stem cavernous angiomas in infants are discussed.     

Further evidence for central histamine H2-receptor involvement in the hypotensive effect of clonidine in the rat

Arteriosclerotic aneurysms of the abdominal aorta constitute a common clinical entity. Rarely are they associated with retroperitoneal fibrosis and ureteral obstruction requiring ureterolysis. Fifteen such cases have been reported, with resection successful in 5 of 7. A sixteenth case is presented complicated by the presence of a persistent left cardinal vein. It is the third aneurysm resected with such an anomaly, and to our knowledge the first to be associated with retroperitoneal fibrosis and ureteral obstruction. Ureterolysis with resection of the aneurysm was performed. The difficulties presented by these pathologic entities, as well as the anomalous venous pattern, are reviewed. Complete preoperative evaluation, including intravenous pyelogram, retrograde pyelography, aortography, and venacavography, for the definition of anatomic relationships and planning of the surgical approach is stressed.     

Sensory and objective characteristics of broiler meat from commercial broilers fed rendered whole-hen meal

A 40-year-old Japanese man with psoriatic arthritis (PA) involving the spine, sacroiliac and peripheral joints presented with dyspnoea and ankle oedema. Blood pressure was 180/110 and 114/80 mmHg in the right and left upper arms, respectively. Examinations showed left ventricular dilatation and diffuse hypokinesis of the left ventricle, with no involvement of the coronary arteries. Aortography detected total occlusion of the left subclavian artery and stenosis of the origin at the right renal artery. Dilated cardiomyopathy and Takayasu's arteritis associated with PA was diagnosed. A few cases of PA have been reported in association with cardiovascular diseases, but the association of these three diseases has not been documented in the literature to date. Dermatologists need to be aware of cardiovascular manifestations in patients with PA, because cardiovascular diseases are not rare in other seronegative spondyloarthropathies.     

Pneumonitis and pulmonary abscess associated with Moraxella nonliquefaciens

A 78-year-old man with pneumonitis and pulmonary abscess associated with Moraxella nonliquefaciens is presented. This organism was found by culture of both transtracheal aspirate and sputum. No previous reports have associated M nonliquefaciens with infection of the lower respiratory tract, although sinusitis and bronchitis have been reported. Possible predisposing factors in our patient included carcinoma of the larynx, as well as alcohol ingestion and cigarette smoking.     

Prenatal diagnosis of cardiac osteoma: a case report

Primary benign intracardiac tumours in the infant period are rare. We report a case of a cardiac osteoma detected at 17 weeks of gestation. Ultrasonographically, it appeared as a calcified mass with a sharp margin and was associated with hypoplastic right ventricle. The gross and histological findings are presented.     

Right ventricular infarction

Right ventricle infarction (RVI) is not a rare clinical entity. It complicates approximately half of inferolateral myocardial infarctions. Under the term RVI we can find mild, asymptomatic dysfunction of right ventricle and cardiogenic shock as well. RVI is associated with increased mortality and its presence obliged us to qualify patient to a high risk group. Diagnosis is based on clinical signs, electrocardiographic findings, hemodynamic measurements and echographic evaluations. The proper treatment of RVI requires support of right ventricle preload with fluid administration, maintainance of atrio-ventricular synchrony, reduction of right ventricle afterload. Early reperfusion with fibrinolytic therapy and coronary angioplasty should be regarded as the prior methods of treatment RVI. Patients who survive RVI have complete resolution of hemodynamic abnormalities with restoration of proper right ventricle function.     

Hemangiopericytoma of the third ventricle. Case report

The authors present the first reported case of a hemangiopericytoma (HPC) occurring in the third ventricle. Most of these lesions are based in the meninges. There is only one other reported case of an intraventricular HPC; in that case the lesion was found in the lateral ventricle. A 40-year-old right-handed man presented with a 3-month history of headaches. Clinical evaluation, including computerized tomography and magnetic resonance imaging studies, revealed a 1-cm enhancing lesion in the third ventricle. Given the findings on the preoperative imaging studies, the lesion was not consistent with some of the more commonly occurring tumors of the third ventricle, namely colloid cysts. A transcortical approach and resection of the lesion was performed without complication. The final pathological findings were consistent with those of an HPC. Hemangiopericytomas rarely occur in the ventricles and may pose a difficult diagnostic dilemma based on their radiographic and gross appearances, as shown in this case. Because of this difficulty, histological confirmation is required to make a definitive diagnosis. These lesions have a propensity to recur and metastasize in the central nervous system and periphery, thus making the goal of treatment a complete surgical resection followed by postoperative radiation therapy in most cases.     

Primary fibrosarcoma of the right ventricle

Mexican literature has information of two fibrosarcomas in the atria. In the present work the first fibrosarcoma of the present work the first fibrosarcoma of the right ventricle found in Mexico is presented. This case behaved clinically, electrocardiographically, and phonomechanocardiographically like an Ebstein's disease, with the exception that in the phono a giant "a" wave was found. A review is mad of the clinical history, EKG, radiologic, and phono findings, as well as the laboratory analysis and data found in the autopsy.     

Echocardiographic aspects of the aortopulmonary window. Report of a case with angiocardiographic correlations

A case of complex congenital heart disease is presented with diagnosis of; ventricular septal defect, interrupt aortic arch, patent ductus arteriosus and aorticopulmonary window. The latter condition was studied by means of M mode and two-dimensional echocardiographic techniques. The heart was scanned perpendicular to its long axis at the origin of the great arteries. This scan revealed an image of two adjacent circular structures which has been previously described as suggestive of dTGA or Double-outlet right ventricle. The clinical picture discarded the possibility of dTGA, and the atrioventricular relationship established by the cross-sectional echocardiography invalidated the diagnosis of Double-outlet right ventricle. The cause of the echocardiographic morphologies observed are discussed.     

Choroid plexus cyst of the left lateral ventricle with intermittent blockage of the foramen of Monro, and initial invagination into the III ventricle in a child

A cyst of the choroid plexus of the left lateral ventricle with intermittent blockage of the foramen of Monro and initially with invagination of the III ventricle in a child is described. In a 6-week-old boy a ventriculoatrial shunt was implanted for correction of an active asymmetrical hydrocephalus of unknown origin. When he was 3 months of age a water-soluble contrast CT ventriculography revealed a noncolloid cyst localised predominantly in the upper portion of the III ventricle. At that time the ventricular catheter obstructed with choroid plexus was removed; new bilateral catheters in a parieto-occipital region were implanted. In the course of the next 4 years, first the atrial catheter had to be extracted and then the peritoneal catheter was changed, in both cases because of obstruction. Periods of normal life alternated with periods of transient and intermittent symptoms of increased intracranial pressure, papilloedema, and myoclonic jerks. Repeated computed tomography (CT) and magnetic resonance imaging (MRI) showed stabilised hydrocephalus with an enlarged left lateral ventricle. When the boy was 16 years old MRI revealed a choroid plexus cyst in the left lateral ventricle 2 cm in diameter, with a ballvalve type of obstruction of the foramen of Monro. CT stereoendoscopic resection of the wall of a large cyst filled with cerebrospinal fluid was performed, and two additional adnexal small cysts were coagulated using the bipolar coagulator, Diomed 25 laser and scissors; the symptoms then regressed, except for superior bilateral altitudinal anopsia. Light and electron microscopy of the cyst wall is reported. The cyst was composed of collagenic connective tissue lined with a basal lamina lacking in epithelial cells. The preoperative and postoperative MRI are presented. Choroid plexus cysts localised in the anterior part of lateral ventricles are very rare, and all reported cases have been in male patients. According to the literature our case is only the third ever described in a child.     

Colloid cysts of the third ventricle --report of one operated and one autopsied case (author's transl)

Colloid cysts of the third ventricle are considered as a relatively rate benign tumor, consituting approximately 0.5% of the whole brain tumors, but the surgical cases have rarely been reported in Japan. In this paper, two cases of colloid cyst are reported and the pathogenesis, the importance of the clinical diagnosis and dramatic results after complete extirpation was emphasized. In Case 1, a 29 year-old right handed male, who showed definite symptoms of increased intracranial pressure without lateralizing signs, was diagnosed as colloid cyst of the third ventricle, and operated on with dissecting microscope on April 20, 1973. The colloid cyst was totally removed and postoperatively V-A shunt was performed for adhesive arachnoiditis. Patient did well after surgery and has returned to the previous work. In Case 2, a 41 year-old male, who suffered from sudden onset of severe headache and bouts of frequent vomiting, was admitted to Tokyo Wome's Medical College Hospital on October 11, 1974. Right cerebral angiography revealed findings of increased intracranial pressure and third ventricle tumor was suspected. On October 13, progressively disturbed consciousness occurred. Immediate ventricular tap and continuous ventricular drainage could not regain consciousness and patient expired on October 30. In necropsy, colloid cyst to the third ventricle was found. As far as these are concerned, these cysts might be reasonably said as ventricular epithelial origin in view of the operative and histological findings. Some emphasis was also made in terms of dissecting microscope in removing this tumor, completely preserving the ventricular walls as well as the terminal veins under magnification.     

Hemimegalencephaly and pathological left handedness. A case report

INTRODUCTION: Hemimegalencephaly is a disorder of neurone proliferation and migration, associated with excessive growth of all or part of one cerebral hemisphere, and variable degrees of pachygyria, polymicrogyria, neuronal heterotopy and gliosis of white matter. It is considered to be a rare malformation with severe encephalopathy, epilepsy resistant to treatment started early, contralateral hemiparesia and severe retardation of development. However, there may also be milder forms. The syndrome of pathological left handedness is associated with disorders of language, visuo-spatial changes and weakness and hypertrophy of the right limbs, with no family history of left handedness. It occurs in lesions predominantly of the left hemisphere which have occurred in the first six years of life. CLINICAL CASE: A 12 year old girl presented with a syndrome of pathological left-handedness and craniofacial asymmetry of the left hemisphere. MR showed the left ventricle and hemisphere to be of increased size and with focal hyperintensity in the white matter, and at the level of the left parietotemporoccipital region there was a thick cortex with little interdigitation of white and grey matter. CONCLUSIONS: Minor forms of hemimegalencephaly are probably more frequent than is generally believed. A high index of suspicion is necessary to identify them. They should be considered in cases of left handedness, craniofacial or bodily asymmetry, cutaneous hamartomas, persistent electroencephalographic asymmetry and asymmetry on neuroimaging with an enlarged ventricle and homolateral hemisphere. Study of this may give information which is useful for understanding plasticity and mechanisms of recovery and functional compensation of immature nerve tissue.     

Sudden cardiac death in patients with arterial hypertension and left ventricular hypertrophy on antihypertensive therapy

The hypertrophy of the left ventricle in patients with arterial hypertension is an independent risk factor which increases c 9 times the probability of sudden cardiac death. Despite the fact that the incidence of sudden cardiac death in patients with arterial hypertension is low, regarding the high occurrence of hypertension it represents a significant medical problem. The therapy of arterial hypertension is able to decrease the general and cardiovascular mortalities with significant interspecies characteristics of individual antihypertensive drugs, as well as to promote the regression of hypertrophy of the left ventricle. The therapy per se can however increase the risk of cardiovascular complications: until now the complication of the therapy by diuretics rich in potassium and beta-blockers are best distinguished. Calcium antagonists are effective antihypertensive drugs but they do not decrease the total mortality. ACE inhibitors have a marked antihypertensive effect and few adverse effects, but until now there is not a sufficient number of large prospective studies which would definitely confirm the preliminary promising findings. Despite the presented problems the cured patients with arterial hypertension have a substantially better prognosis than patients that are not being cured.     

Conal anatomy in aortic atresia, ventricular septal defect, and normally developed left ventricle

Although aortic valve atresia is usually associated with severe underdevelopment of the mitral apparatus and left ventricle in rare cases of aortic atresia, the left ventricle may be of normal size, or even enlarged. This occurrence seems related to the presence of a significant ventricular septal defect. We have presented the morphologic findings in seven patients with aortic atresia and normally developed left ventricle, (six necropsied patients, and one studied angiocardiographically). Four autopsied patients had conal type ventricular septal defects, characterized in three by conoventricular malalignment. Subaortic atresia in these patients resulted from leftward deviation of the conal septum. One patient with aortic atresia and well-developed left ventricle had a membranous defect, and one patient had a complete A-V canal. The ventricular septal defect in the patients with conoventricular malalignment are very similar to the conal VSD observed in patients with aortic arch interruptions. Although ultimate survival with these uncommon groupings of anomalies necessitates patency of the ductus arteriosus, clinical recognition rests on (1) awareness of its existence, (2) ultrasonography, and (3) selective biventricular and aortic angiography. It is possible that some of these patients might be candidates for ventriculo-aortic reconstitiution.