Atrial Septostomy in Patients with End‐Stage Pulmonary Hypertension. No More Needles but Wires,Energy and Close Anatomical Definition

Objectives

To assess the usefulness of a new approach to atrial septal puncture and septostomy in patients with end‐stage pulmonary hypertension.

Background

Atrial septostomy in end‐stage pulmonary hypertension has high mortality and morbidity rates mainly due to trans‐septal catheterization. New approaches to safety during this technical step are expected.

Methods

Twelve patients with end‐stage pulmonary arterial hypertension (5 males, 7 females, mean age 41, 9 ± 12, 0 years) underwent to balloon atrial septostomy. Intracardiac echography (ICE) was used to localize fossa ovalis while a radiofrequency wire was used to perforate the atrial septum. Then a septostomy was performed by progressive balloon dilatation of atrial septum. Septal perforation was successful at the first attempt in 4 patients and after 5 attempts in a single case, while Bas was successful in all.

Results

Pericardial effusion did not develop in any patient. Complications consisted in transient supraventricular tachyarrhythmia, transient cerebral ischemia and severe hypoxemia with ventricular tachycardia in 3 single patients. In‐procedure death rate was 0%. Systemic cardiac output increased immediately, while systemic O2 saturation decreased significantly in all. Mean follow‐up was 8, 2 ± 3, 8 months. Mortality was 16, 6% (2 patients). NYHA class improved in the rest of patients. Four patients (33, 2%) underwent to pulmonary transplant successfully.

Conclusions

This novel approach for trans‐septal catheterization has shown very low rate of major complication during atrial septostomy in patients with end‐stage pulmonary arterial hypertension. (J Interven Cardiol 2013;26:62–68)

     

Clinical use of blade atrial septostomy

A cardiac catheter enclosing an extensible blade was used to enlarge the interatrial opening in seven patients. Two patients with transposition of the great arteries who had balloon atrial septostomy as newborns subsequently presented with clinical evidence of a restrictive interatrial opening at 1 and 4 months of age. Cardiac catheterization confirmed restenosis of the interatrial opening and inadequate intracardiac mixing. After blade atrial septostomy the systemic arterial oxygen saturation increased by 20% and 30%, respectively. Five patients with mitral atresia complex, ages 2 months-9 1/2 years, had a restrictive interatrial communication and severe pulmonary venous hypertension (mean left atrial pressures ranged from 20-38 mm Hg). Following blade atrial septostomy, the pressure gradient between the atria was almost completely abolished and prompt clinical improvement was observed in each patient. All patients tolerated the procedure without complications. Blade atrial septostomy was a safe, effective procedure for enlarging the interatrial communication in this limited series of patients with an interatrial septum too thick to permit adequate rupture by conventional balloon atrial septostomy.     

Surgical therapies for pulmonary arterial hypertension

Surgical therapies for the treatment of pulmonary arterial hypertension typically are reserved for patients who are deemed to be refractory to medical therapy and have evidence of progressive right-sided heart failure. Atrial septostomy, a primarily palliative procedure, may stave off hemodynamic collapse from right-sided heart failure long enough to permit a more definitive surgical treatment such as lung or combined heart-lung transplantation. This article discusses indications for and results of atrial septostomy and lung and heart-lung transplantation in patients who have pulmonary arterial hypertension.     

Balloon atrial septostomy in end-stage pulmonary hypertension guided by a novel intracardiac echocardiographic transducer.

Blade and balloon atrial septostomy has been used to reduce cardiopulmonary symptoms and as a bridge to lung or heart lung transplant in primary pulmonary hypertension. Due to severe right atrial dilatation and resultant loss of anatomical landmarks, the procedure is technically difficult, and the reported postprocedure mortality rate varies between 5% and 50%. Among others, marked systemic desaturation and systemic hypotension presumably secondary to an excessively large atrial septal defect have been reported as causes of postprocedure death. We report a case where a novel intracardiac catheter-based phased-array 5.5--10 MHz transducer with spectral and color-flow Doppler capabilities was used to assist a balloon atrial septostomy and to obtain hemodynamic data in a patient with end-stage pulmonary hypertension.     

Role of prostaglandin E1 infusion in the management of transposition of the great arteries.

That prostaglandin E₁ can produce an increase in systemic oxygen saturation in patients with cyanotic heart disease and ductus dependent pulmonary blood flow has been well documented. However, its use in complete transposition to increase systemic oxygen saturation by increasing mixing has not been well investigated. Ten newborn infants with angiographic diagnosis of d-transposition of the great arteries and patent ductus arteriosus were studied; 6 had an intact ventricular septum. Prostaglandin E₁ infusion (0.1 μg/kg per min) was started after balloon atrial septostomy because of a persistently low systemic oxygen saturation of 26 ± 12 percent (mean ± standard deviation) and oxygen tension of 17 ± 5 torr. The infusion resulted in an increase in systemic oxygen saturation to 53 ± 19 percent (P < 0.01) and oxygen tension to 30 ± 9 torr (P < 0.001). In 2 of 10 patients, there was no increase in systemic oxygen saturation with the infusion (1 had the infusion before the septostomy and both had the infusion for less than 10 minutes). In 8 of 10 patients, the infusion was continued from 4 to 312 hours (average 98 hours) until a Blalock-Hanlon procedure was performed. Two babies became septic, and one of them died. A third had a transient fever. All children whose prostaglandin E₁ infusion was discontinued before atrial septectomy had a reduction in systemic oxygen saturation to unacceptable levels. Two patients who required infusion (for 5 days) after septectomy were successfully weaned from the drug.It is concluded that dilation of the ductus by prostaglandin E₁ infusion increases pulmonary blood flow (left to right shunt) which in turn favorably influences atrial mixing (left to right shunt) and increases systemic oxygen saturation. Pulmonary blood flow may be further increased by a decrease in pulmonary vascular resistance induced by prostaglandin E₁.     

Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect

A severely restrictive atrial septal defect (ASD) in neonates with hypoplastic left heart syndrome (HLHS) results in pulmonary venous hypertension, pulmonary edema, and intractable hypoxia. Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheterization at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography. Three underwent blade septostomy with 2 procedure-related deaths, and 1 had balloon atrial septostomy (BAS); all 4 died before surgical intervention. Fifteen underwent Brockenbrough atrial septoplasty with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a stent placed across the atrial septum after transatrial needle puncture. In the 16 patients treated with septoplasty or stent, oxygen saturation increased from 50 +/- 4% to 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting transplantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation. Eight of 14 (57%) survived to hospital discharge. Six of 7 (86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in profound cyanosis demands urgent intervention. BAS is frequently unsuccessful and blade septostomy has high mortality. Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, allowing stabilization before reconstructive surgery or while awaiting transplant.     

Blade auricular septostomy

We describe the first case of BAS in our country in a three months old child with transposition of the great arteries, restrictive atrial septal defect (RASD) and intact interventricular septum. When he was 15 days old, we performed a balloon atrial septostomy. He had temporal improvement and six weeks later his cyanosis increased, and a new catheterization showed systemic arterial oxygen saturation of 30%, RASD and an interatrial pressure gradient of 2.1 mmHg (left atrium LA: 3.9 and right atrium RA: 1.8). We decided to perform a new septostomy with Park's blade atrial septostomy catheter. After the procedure the interatrial pressure gradient decreased to 0.2 mmHg (RA: 4.3 and LA: 4.5), the angiography shunt and atrial pressures increased. Five months later the child is alive and the systemic arterial oxygen saturation is 51.3%. The technique, advantages and complications are described.     

Atrial septostomy decreases sympathetic overactivity in pulmonary arterial hypertension

BACKGROUND: We have reported previously that the sympathetic nervous system is activated in patients with pulmonary arterial hypertension (PAH), and that this is only partly explained by a decrease in arterial oxygenation. Possible causes for increased muscle sympathetic nerve activity (MSNA) in patients with PAH include right atrial distension and decreased cardiac output. Both may be improved by atrial septostomy, but this intervention also further decreases arterial oxygenation. In the present study, we wanted to investigate the effect of atrial septostomy on MSNA in patients with PAH. METHODS: We recorded BP, heart rate (HR), arterial O2 saturation (SaO2), and MSNA before and after atrial septostomy in PAH patients (mean [+/- SE] age, 48 +/- 5 years) and in closely matched control subjects. Measurements were also performed after septostomy, while SaO2 was brought to the preprocedure level by supplemental O2 therapy. RESULTS: Compared to the control subjects (n = 10), the PAH patients (n = 11) had a lower mean BP (75 +/- 2 vs 96 +/- 3 mm Hg, respectively; p < 0.001), lower mean SaO2 (92 +/- 1% vs 97 +/- 0%, respectively; p < 0.001), increased mean HR (84 +/- 4 vs 68 +/- 3 beats/min; p < 0.01), and markedly increased mean MSNA (76 +/- 5 vs 29 +/- 2 bursts per minute; p < 0.001). Atrial septostomy decreased mean SaO2 (to 85 +/- 2%; p < 0.001) and mean MSNA (to 69 +/- 4 bursts per minute; p < 0.01), but did not affect HR or BP. Therapy with supplemental O2 did not affect MSNA, BP, or HR. The decrease in MSNA was correlated to the decrease in right atrial pressure (r = 0.62; p < 0.05). CONCLUSIONS: Atrial septostomy in PAH patients decreases sympathetic hyperactivity despite an associated decrease in arterial oxygenation, and this appears to be related to decreased right atrial distension.     

The palliative Mustard operation for double outlet right ventricle or transposition of the great arteries associated with ventricular septal defect, pulmonary arterial hypertension, and pulmonary vascular obstructive disease. A report of eight patients.

Five patients with double outlet right ventricle, ventricular septal defect, pulmonary arterial hypertension and pulmonary vascular obstructive disease and three patients with complete d-transposition of the great arteries, ventricular septal defect, pulonary arterial hypertension and pulmonary vascular obstructive disease underwent an elective Mustard baffle operation. The ventricular septal defect was not closed. A large patent ductus arteriosus was divided in three patients. Seven of the eight patients are alive five to 32 months after surgery; one patient died 11 months after surgery. Cyanosis, dyspnea on exertion, and exercise limitation improved initially in all and has persisted in the survivors. In pre and postoperative hemodynamic studies in four patients, systemic arterial oxygen saturation and effective pulmonary blood flow increased from mean values of 70% to 90% and 1.7/min/m2 to 3.3 L/mon/m2, respectively. Absolute systemic and pulmonary flows, and pressures and resistances, were not significantly altered. Criteria for selection of patients with transposition of the great arteries of double outlet right ventricle who would benefit from a palliative Mustard procedure (Mustard atrial baffle without closure of the ventricular spetal defect) are: 1) severe symptoms; 2) pulmonary arteiral hypertension (75% systemic) with pulmonary vascular obstructive disease; and 3) pulmonary artieral oxygen saturation greater than systemic (ascending aorta) arterial oxygen saturation by approximately 10%.     

Graded balloon atrial septostomy in severe pulmonary hypertension

BACKGROUND: The prognosis of patients with severe primary pulmonary hypertension is poor. The role of balloon atrial septostomy as a palliative procedure in these patients is not well defined. We retrospectively analyzed our data regarding the safety, clinical outcome and survival benefit of graded balloon atrial septostomy in patients with severe pulmonary hypertension. METHODS AND RESULTS: Eleven patients (7 males), aged 6 to 30 years (mean age 16.2+/-8.9 years), with severe pulmonary artery hypertension (mean pulmonary artery pressure of 76+/-16.9 mmHg) and refractory congestive heart failure and/or recurrent syncope underwent balloon atrial septostomy. Graded balloon dilatation under echocardiographic guidance and arterial oxygen saturation monitoring was done in all the patients. Procedure-related mortality was 18.2%. Significant acute hemodynamic improvement was seen in the survivors (pre-balloon atrial septostomy cardiac index 1.88+/-0.48 L/min/m2; post-balloon atrial septostomy cardiac index 2.18+/-0.37 L/min/m2, p<0.009). Patients were followed up for a mean period of 20.3 months after the procedure (range: 3 months-5 years). There was functional improvement and increased exercise tolerance in all the patients for a mean follow-up period of 14.6 months (NYHA functional class 3.62+/-0.69 to 2+/-0.50). The estimated probability of survival in this cohort at 1 year was only 48%; but 7 of 8 patients (87%) who survived the procedure were alive at 1 year. CONCLUSION: We conclude that balloon atrial septostomy improves clinical status, hemodynamic variables and possibly also improves survival in selected patients with severe pulmonary artery hypertension. It remains a definite palliative option for refractory primary pulmonary hypertension. However, the procedure-related risks are high in very sick patients and, therefore, balloon atrial septostomy may be advocated early in the course of the disease.     

Blade and balloon atrial septostomy for left heart decompression in patients with severe ventricular dysfunction on extracorporeal membrane oxygenation

Extracorporeal membrane oxygenation (ECMO) is used as circulatory support or bridge to transplantation in patients with severe left ventricular (LV) dysfunction. Left heart decompression is needed to reduce pulmonary edema, prevent pulmonary hemorrhage, and reduce ventricular distention that may aid in recovery of function. We reviewed our experience from November 1993 to December 1997 with 10 patients having severe LV dysfunction (7 myocarditis, 3 dilated cardiomyopathy) who required circulatory support with ECMO and who underwent left heart decompression with blade and balloon atrial septostomy (BBAS). Patients ranged in age from 1 to 24 years (median, 3 years). Indications for BBAS included left atrial/left ventricular distension (10), pulmonary edema/hemorrhage (9), or severe mitral regurgitation (2). BBAS was performed electively in eight patients and urgently in two patients. BBAS was performed while on ECMO in seven patients and pre-ECMO in three. A femoral venous approach was used in all patients. ECMO patients were fully heparinized. Transseptal puncture was required in nine patients while one patient had a patent foramen ovale. Blade septostomy was performed in all patients. Enlargement of the defect was then performed by stationary balloon dilation in nine and Rashkind balloon atrial septostomy in one. Balloon diameters ranged from 10 to 20 mm. Sequential balloon inflations were performed in some patients. Adequacy of the atrial septal defect (ASD) was confirmed by pressure measurement and echocardiography. Adequate left heart decompression was achieved in all patients. Pulmonary edema improved in nine of nine patients. Left atrial mean pressure fell from a mean of 30.5 mm Hg, (range, 12–50 mm Hg) to 16 mm Hg (range, 9–24 mm Hg). Left atrial to right atrial pressure gradient fell from a mean of 20 mm Hg pre-BBAS to 3 mm Hg post-BBAS. ASDs ranged in size from 2.5 to 8 mm (mean, 5.9 mm). Complications included needle perforation of the left atrium without hemodynamic compromise (one), ventricular fibrillation requiring defibrillation (one), and hypotension following BBAS which responded to volume infusion (two). Duration of ECMO ranged from 41 hr to 704 hr (mean, 294 hr). Seven patients survived and four patients had recovery of normal LV function. Of those who recovered, two had no ASD at follow-up while two ASDs are patent 14 days and 3 months post-BBAS. Three patients underwent successful cardiac transplantation. Three patients died, all of whom had multisystem organ failure with or without sepsis. A patent ASD was noted at transplant (three) or autopsy (two). No patient required a second BBAS. BBAS alleviates severe left atrial hypertension and pulmonary edema. In addition, BBAS avoids the potential bleeding complications of surgical left heart decompression. Stationary balloon dilation of the atrial septum is an effective alternative to Rashkind balloon septostomy in older patients. BBAS achieves left heart decompression that may permit recovery of LV function or allow extended ECMO support as a bridge to transplant. Cathet. Cardiovasc. Intervent. 46:179–186, 1999. © 1999 Wiley-Liss, Inc.     

Palliation of systemic sclerosis–associated pulmonary hypertension by atrial septostomy

The onset of pulmonary hypertension in patients with systemic sclerosis carries a poor prognosis. Atrial septostomy has been used successfully to palliate end‐stage primary pulmonary hypertension but has not been attempted in other forms of pulmonary vascular disease. We report substantial clinical improvement following atrial septostomy in a patient with systemic sclerosis complicated by severe, isolated pulmonary hypertension. After the procedure, exercise capacity was improved and exertional syncope abolished. We suggest that this procedure should be considered for other patients with this diagnosis.     

Palliation of systemic sclerosis-associated pulmonary hypertension by atrial septostomy

The onset of pulmonary hypertension in patients with systemic sclerosis carries a poor prognosis. Atrial septostomy has been used successfully to palliate endstage primary pulmonary hypertension but has not been attempted in other forms of pulmonary vascular disease. We report substantial clinical improvement following atrial septostomy in a patient with systemic sclerosis complicated by severe, isolated pulmonary hypertension. After the procedure, exercise capacity was improved and exertional syncope abolished. We suggest that this procedure should be considered for other patients with this diagnosis.     

Acute hemodynamic effects of adjustable atrial septal defect closure in the lateral tunnel Fontan procedure

Objectives. This study evaluated the acute hemodynamic changes with atrial septal defect closure in thek postoperative period in patients undergoing the Fontan procedure.Background. The adjustable atrial septal defect is a modification of the Fontan procedure designed to improve cardiac output and reduce systemic venous hypertension during the postoperative period. Limited information is available on the effects of interatrial shunting on the physiology of direct cavopulmonary connection.Methods. In 11 patients (aged 9 months to 14.5 years), the atrial septal defect was closed 8 h to 4.6 days (mean 1.7 days) postoperatively. Indications for closure included mean right atrial pressure <15 mm Hg or arterial oxygen saturation <80%, or both.Results. Data presented are mean values ±1 SD. Mean right atrial pressure was 13.4 ± 3.0 mm Hg on admission to the intensive care unit, 10.0 ± 2.0 mm Hg (p = 0.02) immediately before closure and 11.4 ± 2.8 mm Hg (p = 0.02) after closure. There was a significant decrease in cardiac output, as calculated from arteriovenous oxygen saturation difference (26 ± 9%, p = 0.003), Doppler aortic flow (19 ± 9%, p = 0.0002) and ventricular volumes by two-dimensional echocardiography (20 ± 8%, p = 0.0001). Arterial oxygen saturation increased from 82 ± 5% to 94 ± 4% (p = 0.0001), and arteriovenous oxygen saturation difference increased from 25 ± 8% to 33 ± 9% (p = 0.0001). Systemic oxygen delivery decreased from 727 ± 354 to 655 ± 325 ml/min per m²(p = 0.02). One patient required reopening of the atrial septal defect.Conclusions. These data demonstrate that a controlled right to left atrial shunt improves cardiac output and systemic oxygen delivery and facilitates the postoperative management of patients after the Fontan procedure. Atrial septal defect closure increases systemic saturation to normal values and prevents potential systemic embolization but significantly decreases oxygen delivery and might limit exercise tolerance.     

Restrictive interatrial communication in total anomalous pulmonary venous connection

Restriction to systemic blood flow at the atrial level in total anomalous pulmonary venous connection (TAPVC) may play a role in the early development of congestive heart failure in some patients. Over a 4-year period, 21 patients with TAPVC without extracardiac obstruction presented from 1 day to 10 months of age (mean 2.6 months). Of 21 patients, 17 (81%) presented after 1 month of age with moderate to severe congestive heart failure. In the first week after birth 4 patients presented with minimal symptoms of congestive heart failure. A small patent foramen ovale (3 mm or less in diameter) was found in 19 of 21 patients (90%) by 2-dimensional echocardiography or angiography. Balloon or blade and balloon atrial septostomy was performed in these 19 patients and resulted in significant decreases in mean right to left atrial pressure gradient (from 2.8 to 0.25 mm Hg, p less than 0.001), systolic pulmonary to femoral artery pressure ratio (from 0.80 to 0.60, p less than 0.001), and systemic arterial oxygen saturation (from 84% to 79%, p less than 0.01). One patient had nonfatal complications. Surgery was performed in 19 patients from 2 weeks to 29 months (mean 12 months) after catheterization, with 1 operative death (5% mortality). Four patients required early total correction because of persistent heart failure; 15 had elective surgical repair. One of 2 unoperated patients died of pneumonia at 2.5 years of age. A restrictive interatrial communication develops after the first month of extrauterine life in most patients with TAPVC. Atrial septostomy results in improved hemodynamic conditions and clinical palliation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Bidirectional shunt in uncomplicated atrial septal defect

The presence of right to left shunts at atrial level in 40 patients with an uncomplicated atrial septal defect was determined by measuring the pulmonary vein to systemic artery oxygen stepdown . In six patients (group 1) a sizeable right to left shunt was found: left atrial oxygen stepdown was greater than or equal to 0.7 vol%, mean right to left shunt 0.67 1/min/m2 (range 0.36-1.0), and arterial oxygen saturation between 84% and 90.5%. The patients in group 1 did not show any differences from those with left to right shunts alone (group 2) as regards sex, cardiac rhythm, heart rate, "a" wave and mean right atrial pressure, end diastolic right ventricular pressure, morphology of diastolic right ventricular pressure curves, pulmonary to systemic vascular resistance ratio, size of the defect, and coexistence of anomalous pulmonary venous drainage. Patients with coexisting right to left shunts were, however, significantly older and had smaller left to right shunts. Thus an appreciable number of patients with uncomplicated atrial septal defects have major right to left shunts which are unrelated to pulmonary hypertension or right heart failure. These shunts may be detected by the usual oximetric techniques and apparently develop with age, which suggests that they result from changes associated with chronic right volume overload.     

Atrial septostomy for pulmonary hypertension

Atrial septostomy represents an additional, promising strategy in the treatment of severe PPH. Experience with this procedure still is limited; however, based on analyses of the worldwide experience, several general conclusions and recommendations can be made. 1. Atrial septostomy can be performed successfully in selected patients with advanced pulmonary vascular disease. 2. Patients with primary pulmonary hypertension who have undergone successful AS have shown: a significant clinical improvement beneficial and long-lasting hemodynamic effects at rest a trend toward improved survival 3. The procedure-related mortality of the collective experience is high (16%). Several recommendations can be made to minimize the risk: [figure: see text] Atrial septostomy should be attempted only in institutions with an established track record in the treatment of advanced pulmonary hypertension, where septostomy is performed with low morbidity. Atrial septostomy should not be performed in patients in whom death is impending or who have severe right ventricular failure and are on maximal cardiorespiratory support. An mRAP greater than 20 mm Hg, PVR index greater than 55 u/m2, and a predicted 1-year survival less than 40% are significant predictors of procedure-related death. Before cardiac catheterization, patients should have an acceptable baseline systemic oxygen saturation (> 90% in room air) and optimized cardiac function (adequate right heart filling pressure, additional inotropic support if necessary). During cardiac catheterization, the following are mandatory: Supplemental oxygen Mild sedation to prevent anxiety Careful monitoring of variables (left atrial pressure, SaO2, and mRAP) Step by step procedure After AS, it is important to optimize oxygen delivery. Transfusion of packed red blood cells or erythropoietin (before and following the procedure, if needed) may be necessary to increase oxygen content. 4. Because the disease process in PPH is unaffected by the procedure (late deaths), the long-term effects of an AS must be considered to be palliative. 5. Despite its risk, AS may represent a viable alternative for selected patients with severe PPH. Indications for the procedure may include: Recurrent syncope or right ventricular failure, despite maximal medical therapy, including oral calcium-channel blockers or continuous intravenous prostacyclin (Fig. 11) As a bridge to transplantation When no other option exists.     

Bidirectional shunt in uncomplicated atrial septal defect.

The presence of right to left shunts at atrial level in 40 patients with an uncomplicated atrial septal defect was determined by measuring the pulmonary vein to systemic artery oxygen stepdown . In six patients (group 1) a sizeable right to left shunt was found: left atrial oxygen stepdown was greater than or equal to 0.7 vol%, mean right to left shunt 0.67 1/min/m2 (range 0.36-1.0), and arterial oxygen saturation between 84% and 90.5%. The patients in group 1 did not show any differences from those with left to right shunts alone (group 2) as regards sex, cardiac rhythm, heart rate, "a" wave and mean right atrial pressure, end diastolic right ventricular pressure, morphology of diastolic right ventricular pressure curves, pulmonary to systemic vascular resistance ratio, size of the defect, and coexistence of anomalous pulmonary venous drainage. Patients with coexisting right to left shunts were, however, significantly older and had smaller left to right shunts. Thus an appreciable number of patients with uncomplicated atrial septal defects have major right to left shunts which are unrelated to pulmonary hypertension or right heart failure. These shunts may be detected by the usual oximetric techniques and apparently develop with age, which suggests that they result from changes associated with chronic right volume overload.     

Atrial septostomy using a butterfly stent in a patient with severe pulmonary arterial hypertension.

We describe a patient with severe pulmonary artery hypertension and refractory right heart failure who underwent "butterfly" stent atrial septostomy guided by intracardiac ultrasound. This technique may be superior to previously reported blade and balloon septostomy because it allows creation of an atrial septal defect of a precise predetermined diameter. The patient's systemic blood flow and clinical status improved significantly after the procedure, allowing her to be accepted as a candidate for lung transplantation.     

Insertion of a fenestrated Amplatzer atrial septostomy device for severe pulmonary hypertension

Atrial septostomy is a palliative treatment for severe pulmonary hypertension. We report the insertion of a novel custom-made fenestrated Amplatzer atrial septostomy device following repeat atrial septostomy for severe pulmonary hypertension in a terminally ill patient with scleroderma resulting in 6 months of palliation.