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CARDIOLOGISTS IN DEVARABESANAHALLI BANGALORE Complex congenital heart disease: Fontan repair Anatomy and physiology Babies with a single ventricle or equivalent defect are often treated during childhood with a palliative operation to connect venous return directly to the pulmonary arteries—a Fontan repair. The prognosis is usually good: 75% of these patients survive at least 20 years. Complications Problems develop because of failure of the systemic ventricle, obstruction of the venous to pulmonary connection, atrial enlargement and AV valve regurgitation. Atrial arrhythmias become increasingly common. Sinus node dysfunction may necessitate pacing, which requires an epicardial electrode in most cases. Follow-up Echocardiography allows assessment of ventricular function and the AV valve. Obstruction in the Fontan connections can be examined with Doppler. MRI is increasingly useful for this assessment. Treatment Severe AV valve regurgitation, cyanosis and ventricular dysfunction are indications for intervention, including transplant or revision of the Fontan. Atrial arrhythmias can be treated with radiofrequency ablation. Pregnancy and contraception Pregnancy is possible in some patients with excellent Fontan function, good LV function and minimal AV valve regurgitation. Maternal risk is high if the Fontan is failing. There may be problems with the need to withdraw ACE inhibitors and with anticoagulation.
Echocardiologist in Kattigenahalli, Bangalore • Pulmonary hypertension Treatment of pulmonary hypertension that is secondary to an underlying respiratory or cardiac condition begins with an attempt to optimise treatment or fix the underling condition. 1 COPD: bronchodilators, steroids, continuous oxygen. 2 Pulmonary fibrosis: oxygen. Aggressive treatment of an underlying connective tissue disease may halt or slow progression of the pulmonary pressures. 3 Pulmonary embolus: anticoagulation, vena caval filter and occasionally pulmonary embolectomy. 4 Mitral stenosis: valvotomy or replacement. 5 Mitral regurgitation: repair or replacement if left ventricular function remains reasonable. 6 Atrial septal defect: surgery or, if suitable, closure in the catheter laboratory, for example with an Amplatzer closure device. There must be evidence of reversibility of the pulmonary pressure if it is nearly as high as systemic pressure, otherwise closure will not improve the prognosis. 7 Eisenmenger’s syndrome: repair of the defect responsible for the shunt is not usually possible once reversal of shunting has occurred. Consider heart and lung transplant if conservative treatment (diuretics, digoxin and sometimes ACE inhibitors) has failed. General measures include continuous oxygen, diuretics, digoxin and aldactone for problems with right heart failure. Patients with pulmonary hypertension that is idiopathic or secondary to connective tissue disease can now be treated with bosentan. This drug is an endothelin receptor antagonist. Endothelin 1 is a potent vasoconstrictor whose levels have been shown to be elevated in patients with idiopathic pulmonary hypertension. Moderate or better improvements in functional capacity and pulmonary artery pressures have been demonstrated after treatment for these patients. The drug is available only for patients with class III symptoms and right atrial pressure greater than 8 mmHg. Patients must demonstrate stable or improved symptoms (usually via a six-minute walk test) for treatment to continue. The drug is very expensive. Its side effects include teratogenicity, an increase in liver enzymes and possibly male infertility. Prostacyclin analogues such as iloprost, which is taken by inhalation, can be effective. Similar restrictions apply to their prescription because of their cost. Continuous intravenous epoprostenol is available in some countries and has been shown to improve symptoms and prognosis in a number of small randomised trials for at least idiopathic pulmonary hypertension patients. Sildenafil (a phosphodiesterase inhibitor) is the most recent vasodilator to become fashionable for idiopathic pulmonary hypertension. A long-acting form has recently become available.
Echocardiologist in Kattigenahalli, Bangalore • Pulmonary hypertension Treatment of pulmonary hypertension that is secondary to an underlying respiratory or cardiac condition begins with an attempt to optimise treatment or fix the underling condition. 1 COPD: bronchodilators, steroids, continuous oxygen. 2 Pulmonary fibrosis: oxygen. Aggressive treatment of an underlying connective tissue disease may halt or slow progression of the pulmonary pressures. 3 Pulmonary embolus: anticoagulation, vena caval filter and occasionally pulmonary embolectomy. 4 Mitral stenosis: valvotomy or replacement. 5 Mitral regurgitation: repair or replacement if left ventricular function remains reasonable. 6 Atrial septal defect: surgery or, if suitable, closure in the catheter laboratory, for example with an Amplatzer closure device. There must be evidence of reversibility of the pulmonary pressure if it is nearly as high as systemic pressure, otherwise closure will not improve the prognosis. 7 Eisenmenger’s syndrome: repair of the defect responsible for the shunt is not usually possible once reversal of shunting has occurred. Consider heart and lung transplant if conservative treatment (diuretics, digoxin and sometimes ACE inhibitors) has failed. General measures include continuous oxygen, diuretics, digoxin and aldactone for problems with right heart failure. Patients with pulmonary hypertension that is idiopathic or secondary to connective tissue disease can now be treated with bosentan. This drug is an endothelin receptor antagonist. Endothelin 1 is a potent vasoconstrictor whose levels have been shown to be elevated in patients with idiopathic pulmonary hypertension. Moderate or better improvements in functional capacity and pulmonary artery pressures have been demonstrated after treatment for these patients. The drug is available only for patients with class III symptoms and right atrial pressure greater than 8 mmHg. Patients must demonstrate stable or improved symptoms (usually via a six-minute walk test) for treatment to continue. The drug is very expensive. Its side effects include teratogenicity, an increase in liver enzymes and possibly male infertility. Prostacyclin analogues such as iloprost, which is taken by inhalation, can be effective. Similar restrictions apply to their prescription because of their cost. Continuous intravenous epoprostenol is available in some countries and has been shown to improve symptoms and prognosis in a number of small randomised trials for at least idiopathic pulmonary hypertension patients. Sildenafil (a phosphodiesterase inhibitor) is the most recent vasodilator to become fashionable for idiopathic pulmonary hypertension. A long-acting form has recently become available.
Echocardiologist in Kattigenahal bangalore Pulmonary hypertension Treatment of pulmonary hypertension that is secondary to an underlying respiratory or cardiac condition begins with an attempt to optimise treatment or fix the underling condition. 1 COPD: bronchodilators, steroids, continuous oxygen. 2 Pulmonary fibrosis: oxygen. Aggressive treatment of an underlying connective tissue disease may halt or slow progression of the pulmonary pressures. 3 Pulmonary embolus: anticoagulation, vena caval filter and occasionally pulmonary embolectomy. 4 Mitral stenosis: valvotomy or replacement. 5 Mitral regurgitation: repair or replacement if left ventricular function remains reasonable. 6 Atrial septal defect: surgery or, if suitable, closure in the catheter laboratory, for example with an Amplatzer closure device. There must be evidence of reversibility of the pulmonary pressure if it is nearly as high as systemic pressure, otherwise closure will not improve the prognosis. Eisenmenger’s syndrome: repair of the defect responsible for the shunt is not usually possible once reversal of shunting has occurred. Consider heart and lung transplant if conservative treatment (diuretics, digoxin and sometimes ACE inhibitors) has failed
Echocardiologist in Kattigenahal bangalore Pulmonary hypertension Treatment of pulmonary hypertension that is secondary to an underlying respiratory or cardiac condition begins with an attempt to optimise treatment or fix the underling condition. 1 COPD: bronchodilators, steroids, continuous oxygen. 2 Pulmonary fibrosis: oxygen. Aggressive treatment of an underlying connective tissue disease may halt or slow progression of the pulmonary pressures. 3 Pulmonary embolus: anticoagulation, vena caval filter and occasionally pulmonary embolectomy. 4 Mitral stenosis: valvotomy or replacement. 5 Mitral regurgitation: repair or replacement if left ventricular function remains reasonable. 6 Atrial septal defect: surgery or, if suitable, closure in the catheter laboratory, for example with an Amplatzer closure device. There must be evidence of reversibility of the pulmonary pressure if it is nearly as high as systemic pressure, otherwise closure will not improve the prognosis. Eisenmenger’s syndrome: repair of the defect responsible for the shunt is not usually possible once reversal of shunting has occurred. Consider heart and lung transplant if conservative treatment (diuretics, digoxin and sometimes ACE inhibitors) has failed
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