Julio Sandoval

Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment.

OBJECTIVES We sought to investigate the acute hemodynamic effects of graded balloon dilation atrial septostomy (BDAS) and to define the long-term impact of this procedure on New York Heart Association functional class and survival in adult patients with primary pulmonary hypertension (PPH). BACKGROUND Current treatment strategies for patients with severe and refractory PPH are limited by either technical difficulties and high mortality or cost. METHODS We studied 15 patients with severe PPH. BDAS was successfully performed in all patients by crossing the interatrial septum with a Brockenbrough needle, followed by progressive dilation of the orifice with a Mansfield balloon in a hemodynamically controlled, step-by-step manner. RESULTS BDAS caused an immediate significant fall in right ventricular end-diastolic pressure and in systemic arterial oxygen saturation and an increase in cardiac index. One patient died, and 14 survived the procedure and significantly improved their mean functional class (from 3.57 +/- 0.6 to 2.07 +/- 0.3 [mean +/- SD], p < 0.001). Exercise endurance (6-min test) also improved from 107 +/- 127 to 217 +/- 108 m (p < 0.001). Because of spontaneous closure, BDAS was repeated in four patients. The survival rate among patients who survived the procedure was 92% at 1, 2 and 3 years, which is better than that for historical control PPH patients (73%, 59% and 52%, respectively). CONCLUSIONS With careful monitoring, BDAS is a safe and useful palliative treatment for selected patients with severe PPH.

Interventional and surgical modalities of treatment in pulmonary hypertension.

Most patients with chronic thromboembolic pulmonary hypertension are operable, and pulmonary endarterectomy is the treatment of choice. Pulmonary endarterectomy should not be delayed for medical therapy, and risk stratification helps to define patients likely to achieve the best outcome. Inoperable patients should be referred for trials of medical agents. Atrial septostomy is promising but underutilized, although better ways of ensuring an adequate, lasting septostomy still need to be determined. Indications for the procedure are unchanged, and it should be considered more frequently. Bilateral sequential lung or heart-lung transplantation is an important option for selected patients, and potential candidates who are class IV or III but not improving should be referred early to a transplantation center. Currently, there is a need for right ventricular assist devices with flow characteristics suited to the circulation of patients with pulmonary arterial hypertension. Right ventricular synchronization therapy has not yet been tested. Novel shunts (e.g., Potts anastomosis) also hold promise. All surgery for pulmonary hypertension should be performed in centers with experience in these techniques.

Primary pulmonary hypertension in children: Clinical characterization and survival

OBJECTIVES This study characterized mortality in a group of Mexican children (n = 18, mean [+/- SD] age 9.9 +/- 3 years) with primary pulmonary hypertension and investigated the factors associated with their survival. BACKGROUND Primary pulmonary hypertension is a progressive, fatal disease of unknown cause. Establishing the diagnosis earlier in life may influence prognosis. METHODS A dynamic cohort of children with primary pulmonary hypertension were enrolled between December 1977 and May 1991 and followed up through September 1992. Measurements included hemodynamic and pulmonary function variables in addition to demographic data, medical history and response to vasodilator treatment. We also compared the survival estimates of these children with those of our adult patients with primary pulmonary hypertension (n = 42, mean age 27.9 +/- 8.5 years). RESULTS Baseline mean (+/- SD) pulmonary artery pressure was similar in children and adults (66 +/- 15 vs. 65 +/- 18 mm Hg, p = NS), but a higher cardiac index resulted in a lower mean pulmonary vascular resistance index in children (18 +/- 7 vs. 26 +/- 12 U/m2, p < 0.01). The proportion of patients who had a positive hemodynamic response to vasodilator treatment was higher in children than in adults (41% vs. 25%). Estimated median survival in children was 4.12 years (95% confidence interval [CI] 0.75 to 8.66) and 3.12 years in adults (95% CI 0.5 to 13.25, chi-square log-rank 0.81, p = NS). Elevated right atrial pressure (rate ratio 10.2) and decreased stroke volume index (rate ratio 32.9) were the only significant predictors of mortality (Cox proportional hazards model). CONCLUSIONS Children with primary pulmonary hypertension have a poor survival expectancy, which does not appear to differ from that in adults with primary pulmonary hypertension. Mortality in childhood primary pulmonary hypertension is also associated with variables that assess right ventricular dysfunction.

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.

Combined Clot Fragmentation and Aspiration in Patients With Acute Pulmonary Embolism

BACKGROUND Massive angiographic pulmonary embolism (PE) with right ventricular dysfunction (RVD) is associated with a high early mortality rate. The therapeutic alternatives for this condition include thrombolysis, surgical embolectomy, or percutaneous mechanical thrombectomy (PMT). We describe our experience using PMT in patients with massive PE and RVD with unsuccessful thrombolysis, increased bleeding risk, or major contraindications for thrombolytic therapy. METHODS Clinical, hemodynamic, and angiographic parameters prior to and following PMT were evaluated. Our primary objective was to describe the incidence of in-hospital cardiovascular death, and of major and minor complications. Mid-term outcomes included analysis of occurrence of cardiovascular death, recurrent pulmonary embolism, change of New York Heart Association functional class, and hospital readmission. RESULTS From July 2004 to May 2007, 69 patients were referred to the cardiac catheterization laboratory with a diagnosis of acute PE, 18 of whom met the criteria for massive PE and are the subject of this study. All patients underwent thrombus fragmentation using a pigtail catheter that was complemented in 13 patients with thrombus aspiration. A percutaneous thrombectomy device (Aspirex; Straub Medical; Wangs, Switzerland) was used in 11 patients. Hemodynamic, angiographic, and blood oxygenation parameters improved after the procedure. A significant increase was observed for systolic systemic BP (74.3+/-7.5 mm Hg vs 89.4+/-11.3 mm Hg, p=0.001) [mean+/-SD], as was a decrease in mean pulmonary artery pressure (37.1+/-8.5 mm Hg vs 32.3+/-10.5 mm Hg , p=0.0001). The in-hospital major complications rate was 11.1%; one patient died from refractory shock, and one patient had intracerebral hemorrhage with minor neurologic sequelae. No cardiovascular deaths or recurrent pulmonary thromboembolism were documented during clinical follow-up (12.3+/-9.4 months). CONCLUSIONS In patients with massive PE, RVD and major contraindications to thrombolytic therapy, increased bleeding risk, failed thrombolysis, or unavailable surgical thrombectomy, PMT appears to be a useful therapeutic alternative.

Pulmonary Embolism as a Cause of Death in Patients With Heart Disease: An Autopsy Study

STUDY OBJECTIVE To establish the prevalence of pulmonary embolism (PE) in autopsy material at a tertiary cardiac referral center and its importance as a cause of death in patients with heart disease (HD). DESIGN Case series. SETTING National Heart Institute, Mexico City. PATIENTS One thousand thirty-two patients who died at our institution from 1985 to 1994 in whom an autopsy study was performed. MEASUREMENTS AND RESULTS Of the 1,032 autopsies reviewed, 231 cases (24.4%) of PE were found; 100 of these patients had a diagnosis of massive PE. Massive PE (obstruction of either of the main pulmonary arteries or more than two lobar arteries) was found to be the third cause of death in this HD population. By age-group distribution, the global prevalence of massive events was higher in patients < 10 years old. Clinical suspicion (premortem) was raised in only 18% of the cases. CONCLUSIONS PE was a frequent cause of morbidity and mortality in patients with HD who underwent autopsies. The incidence of massive PE was high in children.

Original Research: Pulmonary EmbolismPulmonary Embolism as a Cause of Death in Patients With Heart Disease: An Autopsy Study

STUDY OBJECTIVE To establish the prevalence of pulmonary embolism (PE) in autopsy material at a tertiary cardiac referral center and its importance as a cause of death in patients with heart disease (HD). DESIGN Case series. SETTING National Heart Institute, Mexico City. PATIENTS One thousand thirty-two patients who died at our institution from 1985 to 1994 in whom an autopsy study was performed. MEASUREMENTS AND RESULTS Of the 1,032 autopsies reviewed, 231 cases (24.4%) of PE were found; 100 of these patients had a diagnosis of massive PE. Massive PE (obstruction of either of the main pulmonary arteries or more than two lobar arteries) was found to be the third cause of death in this HD population. By age-group distribution, the global prevalence of massive events was higher in patients < 10 years old. Clinical suspicion (premortem) was raised in only 18% of the cases. CONCLUSIONS PE was a frequent cause of morbidity and mortality in patients with HD who underwent autopsies. The incidence of massive PE was high in children.

Does Anticoagulation in Eisenmenger Syndrome Impact Long‐term Survival?

OBJECTIVE To determine the impact of anticoagulation on survival in Eisenmenger syndrome. BACKGROUND The use of anticoagulation for primary prevention of adverse events in patients with Eisenmenger syndrome has been proposed but not studied. Strong arguments have been made both for and against anticoagulation based on the known risk of hemoptysis and pulmonary vascular thrombosis. DESIGN AND SETTING Retrospective cohort study at a tertiary referral hospital. PATIENTS AND INTERVENTIONS   One hundred forty-four patients with established Eisenmenger physiology all underwent initial laboratory, echocardiographic, and catheterization evaluation after initial referral. We retrospectively identified patients who were started on anticoagulation (AC) and compared them to patients who did not receive anticoagulation therapy (non-AC). Baseline variables were compared between groups, as well as between survivors and nonsurvivors. Analyses of prognostic factors and survival were done using Cox and Kaplan-Meier methods. OUTCOME MEASURES The primary outcome was death since time of baseline evaluation. RESULTS We identified 48 anticoagulated and 44 non-anticoagulated patients with Eisenmenger physiology (oxygen saturation 82 ± 9%, PaO(2) 48 ± 8 mm Hg, hemoglobin 18.6 ± 4 g/dL). More atrial septal defect patients were in the AC group, but there were no other baseline differences in clinical, functional, or hemodynamic data. After mean follow-up of 7 ± 5.4 years (range 1-31), 11 patients died in the AC and 10 died in the non-AC group. There was no survival difference between groups (log rank test = 1.78; P is not significant). For the entire cohort, mortality was significantly associated with New York Heart Association class 3-4 (hazard ratio = 4.2), evidence of right heart failure (hazard ratio = 13.6), and a mean corpuscular volume <80 fL (hazard ratio = 3.8). Use of anticoagulation did not impact survival. Bleeding complications occurred in seven (16%) of AC patients, including two fatalities. CONCLUSIONS Anticoagulation had no impact on long-term survival in this limited study. These data may be useful in considering future studies addressing this question.

Atrial septal defect in adults ≥40 years old: negative impact of low arterial oxygen saturation

BACKGROUND Although good prognosis and clinical long-term outcome have been commonly reported in minimally symptomatic adult patients with ASD, this information has been based on studies with a relatively small number of adult patients. We studied unoperated patients aged over 40 years to define the patterns of presentation, anatomical characteristics, outcome and predictive factors for free-event survival of major cardiovascular and pulmonary events. METHODS AND RESULTS Two-hundred survivors of atrial septal defect aged over 40-yr attended from 1985 to 1998 were reviewed and followed-up from 1.6 to 22 years. Patients were classified in three groups according to age at entry: Group 1, between 40 and 49; Group 2; 50 and 59; and Group 3, > or =60 years old. The mean age at presentation was 48.8+/-9.2 years, and the most common clinical presentations were arrhythmia and dyspnea (51.4%). There were 37 (18.5%) events: 7 heart failure-related, 5 sudden death, 13 severe pulmonary infections, 5 embolisms, and 4 strokes. According to Coxs regression analysis, predictors of primary end point included age group at presentation (hazard ratio 1.71, 95% confidence limits 1.16 to 2.54), and either pulmonary hypertension (mean pulmonary pressure >35 mmHg; hazard ratio=0.65 (4.6, confidence limits 2.2 to 9.5) or, arterial oxygen saturation <80% (hazard ratio 1.71, 95% confidence limits 1.16 to 2.54). CONCLUSIONS This study supports that long term outcome of patients aged >40 years with unoperated ASD is importantly determined by the mPAP (>35 mmHg), SaO2% (_80) and the age at diagnosis. Nevertheless we identified an inverse association between the mPAP level and SaO2% (interaction). The event-free survival expectancy may be estimated using the age at diagnosis and either SaO2% or mPAP. This prognostic stratification based on pathophysiological principles, may help in making decisions for therapeutic interventions. SaO2% should always be measured as a part of the initial clinical approach of those patients with atrial septal defect aged over 40 years.

Echocardiographic Evaluation of Patients with Primary Pulmonary Hypertension Before and After Atrial Septostomy.

Objectives: To characterize the early changes in right ventricular [right ventricle (RV)] geometry and function, as assessed by two‐dimensional (2‐D) and Doppler echocardiography, after balloon‐dilation atrial septostomy (BDAS) in patients with severe primary pulmonary hypertension (PPH). Background. Survival in PPH is to a great extent dependent on the functional status of the RV. BDAS recently has been shown to improve functional class and hemodynamics in patients with PPH nonresponsive to conventional vasodilator treatment. Methods: Ten patients with severe PPH who underwent BDAS were studied with transthoracic and transesophageal 2‐D and Doppler echocardiography. RV dimensions were measured in the apical four‐chamber view. Continuous‐wave Doppler echocardiography was used to obtain peak velocity of tricuspid regurgitation. Transesophageal echocardiography (TEE) primarily was used for the follow‐up of the atrial septal defects (ASDs). Results: In the early post‐BDAS studies, right atrial and ventricular dimensions significantly decreased in all patients (P < 0.05). Global right ventricular wall motion (RVWM) also improved. RV percent change in area after septostomy inversely correlated with the changes in RV systolic area (r =–0.75; P < 0.05) and also with the baseline (preprocedure) values of RV percent change in area (r =–0.77; P < 0.05). Neither RV wall thickness nor the degree of tricuspid regurgitation were modified significantly after the procedure. Conclusions: BDAS in the setting of severe PPH results in moderate and salutary changes in geometry and function of the RV as assessed by 2‐D echocardiography. These changes mainly appear to be the result of the decompression effect of atrial septostomy.