1/21. Transient left ventricular failure following bilateral lung transplantation for pulmonary hypertension.BACKGROUND: Bilateral lung transplantation is an established therapy for end-stage pulmonary hypertension. Its early postoperative outcome may be biased by various complications resulting in unexpected deterioration of the patient in terms of hemodynamics and blood gases. methods: We have reviewed the early postoperative course of patients who underwent bilateral lung transplantation for pulmonary hypertension at our institution and analyzed all available data, especially hemodynamic measurements, echocardiographic documentation and therapeutical strategies, in those cases where cardiac dysfunction was found to be responsible for clinical deterioration. RESULTS: Three out of 20 lung transplant recipients operated for pulmonary hypertension experienced severe respiratory insufficiency accompanied by hemodynamic decompensation during the first days after surgery. Clinical and laboratory findings together with results of echocardiography and pulmonary artery catheterism helped establish the diagnosis of left ventricular failure. This proved to be transitory, but the response to therapy (inotropic drugs, afterload reduction and eventually prostaglandins) was very variable. Adequately treated, this complication did not preclude the outcome of transplantation by itself. CONCLUSION: Left ventricular failure is a possible complication after lung transplantation for pulmonary hypertension. echocardiography and pulmonary artery catheterism may be useful adjuvant diagnostic tools, beside routine physical examination, chest X-ray, and laboratory analysis. Therapy of this complication must be adapted individually and may be complex.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/21. pulmonary artery stenosis 5 years after single lung transplantation in primary pulmonary hypertension.This is a case report about a 56-year-old female patient with primary pulmonary hypertension who underwent single, right lung transplantation. Five years postoperatively she developed signs of right heart failure. history and physical examination suggested pulmonary artery stenosis. diagnosis was confirmed by pulmonary angiography. Percutaneous placement of a balloon expandable stent normalized pulmonary artery pressure.- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
3/21. Familial primary pulmonary hypertension and associated ocular findings.BACKGROUND: Familial primary pulmonary hypertension (PPH) is a rare, fatal, autosomal dominant disease that results in right heart failure from idiopathic obliteration of the pulmonary arteries. patients develop stagnation of venous blood flow and elevated venous pressure. methods: The authors retrospectively reviewed the clinical records of three patients diagnosed with PPH that was confirmed on the basis of physical examination, chest X-ray, electrocardiogram, and echocardiogram. cardiac catheterization excluded cardiac shunt and other secondary causes of pulmonary hypertension. RESULTS: Two patients presented with a clinical picture resembling venous stasis retinopathy, and one with bilateral choroidal detachments. Two patients had delayed choroidal filling on fluorescein angiography, which was confirmed in one patient with indocyanine green videoangiography. CONCLUSIONS: Elevated venous pressure found in PPH is responsible for the delayed choroidal perfusion and the reduced venous blood outflow. This explains the clinical findings of venous stasis retinopathy and choroidal detachments seen in these patients.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
4/21. Long term inhalation of iloprost in a child with primary pulmonary hypertension: an alternative to continuous infusion.Primary pulmonary hypertension is a rare disease in childhood associated with a poor prognosis. However, during the past 10 years, pulmonary vasodilator treatment has somewhat improved its prognosis. Long term continuous infusion of prostacyclin (epoprostenol) has been shown to improve physical capacity and to reduce mortality in primary and secondary pulmonary hypertension. It has been reported in adults that daily repetitive inhalation of iloprost, a prostacyclin analogue, seems also suitable for long term therapy of pulmonary hypertension. Repetitive inhalation of iloprost was administered to a 5 year old boy with severe primary pulmonary hypertension. He showed continuous clinical improvement without any side effects over the three years of treatment. This treatment may offer an alternative to continuous intravenous prostacyclin infusion and obviates the need for a permanent central venous catheter.- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
5/21. Long term survival in primary pulmonary hypertension.The mean survival of patients with severe primary pulmonary hypertension (PPH) is < 3 years without appropriate treatment. There are no long term reports on the spontaneous course of mild PPH over a longer period. Stable long term follow up is described of a 39 year old patient with PPH without treatment over a 30 year period. PPH had been diagnosed 30 years previously after right heart catheterisation (mean pulmonary artery pressure 35 mm Hg) and 30 years later, repeated measurements showed nearly unchanged haemodynamic parameters. Further examinations confirmed the diagnosis of PPH. It is suggested that PPH with modestly limited physical activity (new york Heart association functional class II) does not always seem to coincide with progression of the disease and, therefore, it may be feasible to withhold treatment while closely monitoring these patients.- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
6/21. Sildenafil for portopulmonary hypertension in a patient undergoing liver transplantation.liver transplantation (LT) may be indicated in cirrhotic patients with underlying pulmonary artery hypertension. However, severe pulmonary artery hypertension with mean pulmonary artery pressure (mPAP) above 50 mmHg has even been considered a contraindication to LT. We present a cirrhotic patient with an mPAP of 56 mmHg measured using right heart catheterization (RHC) and with severely compromised physical capacity. She was first treated with sildenafil (Viagra), a potent novel vasodilator, and successfully transplanted later. The mPAP decreased with sildenafil to the level of 28-31 mmHg and her general condition improved markedly. An LT using piggyback technique was performed 16 weeks later. Despite 2 reoperations for bleeding, the outcome has been excellent. In conclusion, treatment of severe portopulmonary hypertension (PHT) with sildenafil is effective. If a decrease in mPAP is achieved with sildenafil, it may improve the result of LT, even though no evidence of reversibility of PPHTN exists.- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
7/21. A case of Adams-Oliver syndrome with associated brain and pulmonary involvement: further evidence of vascular pathology?We report on a case of Adams-Oliver syndrome (AOS) with typical skin and limb defects along with the unique findings of pulmonary hypertension and central nervous system (CNS) involvement. Adams-Oliver syndrome has a wide spectrum of physical anomalies ranging from characteristic aplasia cutis congenita (ACC), transverse limb defects, and cutis marmorata telangiectica to extensive lethal anomalies. While pulmonary hypertension is usually not associated with AOS, the abnormal endothelial regulation of vascular tone seen in the pulmonary vasculature may enhance current pathophysiologic concepts of vascular abnormalities in AOS. There is accumulating evidence of significant CNS defects in AOS. This infant had hypoplastic corpus callosum and focal findings in the periventricular white matter. Evaluation for pulmonary hypertension and CNS anomalies in patients suspected to have AOS, can help identify those who are at risk for acute morbidities and associated developmental delays.- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
8/21. Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports.We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
9/21. association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations.To determine the clinical, laboratory and hemodynamic profile in patients with primary pulmonary hypertension and associated portal hypertension, 7 new cases and 71 previously reported cases were analyzed. There was no gender predilection and the average age at diagnosis was 41 years. liver cirrhosis was the most frequent cause of hypertension (82%) and a surgical portosystemic shunt was present in 29%. Almost invariably, portal hypertension either preceded or was diagnosed concurrently with pulmonary hypertension, favoring the hypothesis that in portal hypertension, the pulmonary vasculature may be exposed to vasoactive substances normally metabolized or produced by the diseased liver, possibly inducing vasoconstriction or direct toxic damage to the pulmonary arteries. Clinically, exertional dyspnea was the most frequent presenting symptom (81%); other symptoms, such as syncope, chest pain and fatigue, were present in less than 33%. An accentuated pulmonary component of the second heart sound (82%) and a systolic murmur (69%) were the most common physical findings. At least 75% of these patients had evidence of pulmonary hypertension on electrocardiography (right ventricular hypertrophy) or roentgenography (cardiomegaly or dilated main pulmonary arteries, or both). Hemodynamic findings included severe pulmonary hypertension (mean pulmonary artery pressure 59 /- 19 mm Hg) with normal pulmonary capillary wedge pressure and cardiac output. Treatment was basically palliative and the mean and median survival times were 15 and 6 months, respectively. In brief, on the basis of clinical presentation and laboratory features, patients with combined primary pulmonary hypertension and portal hypertension seldom represent a diagnostic challenge. Further research is needed on treatment, which remains palliative. The survival rate is poor and worse than that seen in isolated primary pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 0.12149266931436keywords = physical (Clic here for more details about this article) |
10/21. Chronic cor pulmonale.Chronic cor pulmonale is defined as right heart hypertrophy and/or chronic right heart failure. There are many etiologies, but the common cause is increased right heart work from pulmonary hypertension. Etiology can be conveniently discussed by assuming two prototypes, the asphyxial or hypoxic type and the vascular obliterative type. A common cause of the asphyxial type is chronic obstructive pulmonary disease, and the obliterative type is represented by chronic pulmonary thromboembolic disease or primary pulmonary hypertension. pathology is discussed, emphasizing the cardiac manifestations of chronic cor pulmonale including data of specific cardiac chamber size. An overview of hemodynamics is given, and the use and limitation of electrocardiography and chest x-rays are discussed. The exciting potential use of echocardiography for the serial non-invasive measurement of anatomical and pathophysiological features is outlined, along with the value of a careful physical examination and the proper utilization of laboratory tests in the diagnosis of chronic cor pulmonale. In the patient with the asphyxial type, the treatment of pulmonary infectious exacerbations, the role of corticosteroids, digoxin, diuretics, phlebotomy, bronchodilators (theophylline, beta adrenergic agonists, and anticholinergics), and long-term oxygen therapy is noted. The controversy surrounding the use of vasodilators and calcium blockers in these patients is discussed. Treatment aspects of the vascular obliterative type, including the role of vasodilators, calcium blockers, prostacyclin, anticoagulants, and overall strategy are discussed. A brief note is mentioned of the promising role of surgical therapy in chronic thromboembolic disease causing chronic cor pulmonale.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
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