1/49. Pulmonary stenosis and severe biventricular dysfunction: improvement following percutaneous valvuloplasty.A 15-year-old boy with severe pulmonary stenosis associated with severe right and left ventricular systolic dysfunction is reported. After successful percutaneous pulmonary valvuloplasty, there was an initial and early improvement in right ventricular (RV) function, followed by a delayed and more gradual improvement in left ventricular (LV) function. At long-term follow up, both RV and LV systolic functions were nearly normalized. Several mechanisms may be implicated, including ventricular interdependence, geometric factors, altered compliance and intrinsic alteration in the LV muscle. A delayed, but sustained, improvement in LV systolic function following relief of RV pressure overload suggests that the latter mechanism must have played an important role in the genesis of the LV dysfunction. Pulmonary stenosis associated with severe biventricular dysfunction may be treated primarily by percutaneous pulmonary balloon valvuloplasty with near-total recovery of the ventricular function.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/49. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure.Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/49. Combined percutaneous atrial septal defect occlusion and pulmonary balloon valvuloplasty in adult patients.Severe pulmonary stenosis in association with a large atrial septal defect is uncommon. When these 2 conditions are present, significant left-to-right shunt is often prevented by the outflow obstruction, which protects the pulmonary bed until adulthood. This report shows our initial experience of percutaneous treatment of both congenital malformations, either staged or combined in the same procedure, in 2 adult patients whose treatments yielded effective atrial septal defect occlusion and right ventricular pressure relief that persisted at mid-term follow-up. Although these opposite procedures (opening and closing) have been applied as isolated methods of treatment, this preliminary experience appears to demonstrate the feasibility and effectiveness of a combined percutaneous treatment.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/49. Balloon pulmonary valvuloplasty in carcinoid syndrome.Half of all patients with carcinoid syndrome develop cardiac involvement. patients who have cardiac involvement have a significantly worse prognosis than those without, and death can occur directly as a result of cardiac involvement. A case of carcinoid syndrome in a 38 year old woman with lesions in the liver, who presented with right sided valvar abnormalities, a dilated right ventricle, and right ventricular pressure overload, is presented. In order to palliate the patient's symptoms and to decrease right sided pressures before major abdominal surgery, balloon pulmonary valvuloplasty was performed at the time of cardiac catheterisation. This resulted in a reduction in the pulmonary gradient and right ventricular pressure. Following the procedure, the patient's symptoms were completely relieved. She went on to laparotomy where the lesions in the liver were excised without complication.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
5/49. Bronchospasm induced by cardiopulmonary bypass.Severe bronchospasm during cardiopulmonary bypass (CPB) is an unusual event. A 16-year-old girl with pulmonary stenosis who underwent reconstruction of the right ventricle outflow tract experienced severe bronchospasm following CPB. Just after the initiation of the partial CPB, high inspiratory airway pressure was suddenly recognized. The lung had become too stiff for the anesthetic circuit bag to be squeezed by hand. Tracheobronchial obstruction was ruled out by investigation with a fiberoptic bronchoscope. A presumptive diagnosis of severe bronchospasm was made, and aggressive bronchodilator therapy was instituted. The attack was successfully treated with aggressive bronchodilator therapy. Although the exact causes for bronchospasm in our case are not clear, CPB factors, such as the release of complements and allergic reactions might have induced the attack under relatively light anesthetic state.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/49. In utero progressive pulmonary stenosis successfully treated with transcatheter intervention after delivery.It is unclear whether pulmonary stenosis with intact ventricular septum is a secondary cardiac malformation. We report an infant with pulmonary stenosis (diagnosed by fetal echocardiography) with progressive obstruction in late gestation who presented with increasing transvalvular pressure gradients (15 mm Hg at 22 weeks' gestation to 47 mm Hg at 35 weeks). The tricuspid/mitral valve annulus ratio decreased from 1.25 at 24 weeks' gestation to 0.96 at 33 weeks. At 38 weeks' gestation, a male infant weighing 3,524 g, with Apgar scores of 9 and 9 at 1 and 5 minutes, respectively, was delivered by cesarean section. Critical pulmonary stenosis was confirmed by postnatal catheterization. These findings support the postulation that pulmonary stenosis is a progressive disorder. After percutaneous balloon dilatation, the transvalvular pressure gradient decreased and the right ventricular cavity increased gradually. The transvalvular pressure gradient had decreased to 15 mm Hg and the tricuspid/mitral valve annulus ratio was 0.93 at the age of 2 years.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
7/49. Three-decade follow-up in pulmonary artery ectasia: risk assessment strategy.We present a 29-year follow-up in a patient with ectasia of the pulmonary trunk and mild valvular stenosis. There was no progression from the first presentation at the age of 15 months until the actual age of 30 years, the z-value of the diameter of the pulmonary trunk remaining almost constant (z-value 22). This indicates that pulmonary dilatation with normal pressure may be a benign disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/49. Exceptional survival of a patient with large ventricular septal defect, bidirectional shunt, and severe pulmonary valve stenosis.A 74-year-old man has survived in good health for an exceptionally long time despite the presence of a moderate-to-large-sized membranous ventricular septal defect (VSD). He has remained acyanotic with new york heart association class I function. Transthoracic and transesophageal echocardiography with color flow Doppler demonstrated a membranous VSD with left-to-right and right-to-left bidirectional shunts during ventricular systole and diastole, respectively, with an right ventricular systolic pressure of 93 mm Hg, dilation of the atria and the right ventricle, and right ventricular hypertrophy. The pulmonary valve was severely stenotic with transpulmonary valve peak velocity of 6.1 m/s and a peak pressure gradient of 147 mm Hg. The pulmonary artery and inferior vena cava were mildly dilated, and the left ventricular dimension and systolic function were normal. Transesophageal echocardiography with saline solution microbubble injection demonstrated positive contrast effect in the left ventricle in diastole confirming a right-to-left shunt at the ventricular level. This man is currently the oldest survivor with a moderate-to-large-sized membranous VSD reported in the literature.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
9/49. Right ventricular outflow tract obstruction due to extrinsic compression by non-Hodgkin's lymphoma: importance of echocardiographic diagnosis and follow up.Acquired right ventricular outflow tract obstruction due to extrinsic compression of the pulmonary artery is a rare manifestation of non-Hodgkin's lymphoma (NHL). We report a case of a 17 year old boy who was referred for evaluation of a large anterior mediastinal mass, causing dyspnea and cough and resulting in a harsh systolic murmur. echocardiography demonstrated compression of the pulmonary artery by the mass, with a severe pressure gradient. biopsy revealed intermediate grade, diffuse large cell NHL. Systemic chemotherapy rapidly led to a significant decrease in the size of the mass, and virtual disappearance of the pressure gradient. In this report, the use of echocardiography for diagnosis and follow up of extracardiac tumors is reviewed. It is suggested that this technique may also be useful for the routine staging of mediastinal lymphomas because of the potential consequences of clinically undetectable hemodynamic compromise.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
10/49. Low pressure giant pulmonary artery aneurysms in the adult: natural history and management strategies.OBJECTIVE: To describe aspects of the natural history and pathophysiology of giant low pressure pulmonary artery aneurysms and to propose potential surgical strategies. DESIGN: Cross sectional retrospective review. SETTING: Supraregional tertiary referral centre. patients: All adult patients referred for assessment of giant pulmonary artery aneurysm retrospectively identified from the Mayo adult Congenital heart disease Clinic database. methods: Patient data were reviewed from hospital records, including echocardiograms, magnetic resonance images, radiographs, and histology slides. RESULTS: Four patients were identified with a median age of 52 years (range 37-64 years). Presenting symptoms were effort related dyspnoea, chest discomfort, and hoarseness in one patient. All patients had pulmonary regurgitation and clinical evidence of right ventricular enlargement in association with a pulsatile mass at the upper left sternal edge. Transthoracic echocardiography showed the giant pulmonary artery aneurysm involving the main pulmonary artery and proximal branches, and confirmed severe pulmonary regurgitation in all patients. None of the patients had intimal tearing, medial dissection, or pulmonary arterial rupture. The pulmonary valve was replaced to relieve symptoms and preserve right ventricular function. Pulmonary arterial histology showed medial degeneration of elastic fibres with accumulation of basophilic ground substance. CONCLUSIONS: rupture or dissection of these low pressure aneurysms is rare. The timing of surgical intervention should be determined by changes in right ventricular size and function resulting from pulmonary regurgitation or pulmonary stenosis, and not the size of the aneurysm.- - - - - - - - - - ranking = 6keywords = pressure (Clic here for more details about this article) |
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