老年先天性心脏病心房间隔缺损患者左心室舒张功能受限的诊断和介入治疗前的预适应治疗

目的:探讨诊断和预防老年(>60岁)房间隔缺损(ASD)患者内科介入治疗后发生急性左心衰竭(心衰)至肺水肿风险的方法和手段。方法:术前进行常规经胸超声心动图检查(TTE),术中先行球囊封堵ASD,测量左心房压力。如果球囊封堵ASD后,左心房平均压升至10 mm Hg(1 mm Hg=0.133kPa)以上时,定义为左心室舒张受限(LVR)。对于此类患者暂停此次介入,并给予左心室预适应治疗(LVPCT)48 h,然后进行第2次介入治疗。术后将有创血流动力学指标与TTE结果进行对比。结果:23例患者,15例患者在第1次介入治疗过程中,没有发现LVR,ASD介入治疗1次完成。8例患者(34.8%)在第1次介入治疗过程中,被诊断LVR。其中6例患者的左心房平均压在经过48 h的LVPCT后显著下降,第2次介入治疗时封闭ASD。其余2例患者在接受48 h LVPCT后,球囊导管测量ASD封堵后左心房平均压仍>10 mm Hg,其中1例患者在术后发生急性左心衰竭。通过与有创血流动力学指标对比,胸骨旁长轴M型超声测量左心室舒张末期直径(LVEDD)≤40 mm和右心室舒张末期直径(RVEDD)≥30 mm合并心室间隔矛盾运动可预见LVR的存在(P<0.05)。结论:术前超声心动图可以预测左心室舒张受限的存在。介入治疗术中使用球囊封堵ASD后测量左心房压有助于诊断LVR。术前静脉LVPCT可以有效预防术后充血性心衰的出现。     

Left ventricular conditioning in the elderly patient to prevent congestive heart failure after transcatheter closure of atrial septal defect.

Transcatheter closure of atrial septal defects (ASDs) is a safe and effective treatment. Over the past years, an increasing number of elderly patients (age > 60 years) have been admitted for transcatheter closure to prevent ongoing congestive heart failure from volume overload. However, recent data point to the risk of serious acute left ventricular dysfunction leading to pulmonary edema immediately after surgical or transcatheter ASD closure in some patients. In this study, we used a technique described before to recognize in advance patients at risk of left heart failure after ASD closure. Those patients at risk were then treated with preventive conditioning medication for 48-72 hr before definitive transcatheter ASD closure was performed. Fifty-nine patients aged over 60 years (range, 60-81.8 years; median, 68 years) were admitted to our institution for transcatheter closure of an atrial septal defect. All patients received evaluation of atrial pressures before and during temporary balloon occlusion of the ASD. Patients with left ventricular restriction due to increased mean atrial pressures (> 10 mm Hg) during ASD occlusion received anticongestive conditioning medication with i.v. dopamine, milrinone, and furosemide for 48-72 hr before definitive ASD closure with an Amplatzer septal occluder was performed. In 44 patients without any signs of left ventricular restriction, ASD closure was performed within the first session. Fifteen (25%) out of 59 patients showed left ventricular restriction. In the majority of patients with LV restriction, the mean left atrial pressures with occluded ASD were significantly decreased after 48-72 hr of conditioning medication. Definitive ASD closure was then performed in a second session. Only two patients received a fenestrated 32 mm Amplatzer occluder due to persistent increased atrial pressures > 10 mm Hg even after conditioning medication. There were no significant differences in shunt, device size, or defect size between the two groups. Balloon occlusion of atrial septal defects identifies patients with left ventricular restrictive physiology before ASD closure. Intravenous anticongestive conditioning medication seems to be highly effective in preventing congestive heart failure after interventional closure of an ASD in the elderly patient with a restrictive left ventricle.     

Left ventricular function in adult patients with atrial septal defect: implication for development of heart failure after transcatheter closure

Despite advances in device closure for atrial septal defect (ASD), post-closure heart failure observed in adult patients remains a clinical problem. Although right heart volume overload is the fundamental pathophysiology in ASD, the post-closure heart failure characterized by acute pulmonary congestion is likely because of age-related left ventricular diastolic dysfunction, which is manifested by acute volume loading with ASD closure. Aging also appears to play important roles in the pathophysiology of heart failure through several mechanisms other than diastolic dysfunction, including ventricular systolic and vascular stiffening and increased incidence of comorbidities that significantly affect cardiovascular function. Recent studies suggested that accurate assessment of preclosure diastolic function, such as test ASD occlusion, may help identify high-risk patients for post-closure heart failure. Anti–heart failure therapy before device closure or the use of fenestrated device appears to be effective in preventing post-closure heart failure in the high-risk patients. However, the long-term outcome of such patients remains to be elucidated. Future studies are warranted to construct an algorithm to identify and treat patients at high risk for heart failure after device closure of ASD.     

Acute left heart failure after interventional occlusion of an atrial septal defect]

Congestive left ventricular failure after surgical closure of an atrial septal defect (ASD) has been repeatedly reported, particularly in the elderly. We present a case of left ventricular failure after a successful transcatheter closure of an ASD, which to our knowledge has not been described before. In a 78-year-old woman (50 kg, 160 cm) with well-preserved left ventricular function (ejection fraction 65%) and without coronary artery disease or arterial hypertension, an ASD (Qp/Qs 1.6:1) was closed with an Amplatzer Septal Occluder without a residual shunt. Two hours after the procedure, she developed pulmonary edema due to left ventricular failure (increase of end-diastolic diameter from 42 mm to 54 mm, ejection fraction 20%), had to be mechanically ventilated for 24 hours and needed catecholamines for 4 days. High doses of diuretics were supplied until the ejection fraction normalized (32%). The patient could not be discharged until two weeks after intervention. A reduced preload for decades may predispose acute left ventricular failure, particularly in the elderly with compromised ventricular compliance.     

Pulsed Doppler assessment of left ventricular diastolic function in atrial septal defect

From January 1986 through December 1990, we used pulsed Doppler echocardiography to evaluate left ventricular diastolic function in 43 patients with an ostium secundum atrial septal defect. The study population included 27 females and 16 males, whose ages ranged from 6 to 58 years (mean, 26 years). The patients were grouped according to degree of pulmonary hemodynamic impairment: patients in Group 1 (n = 6) had severe pulmonary hypertension, those in Group 2 (n = 10) had mild-to-moderate pulmonary hypertension, and those in Group 3 (n = 27) had no pulmonary hypertension. For comparison, we also evaluated 30 healthy individuals. All control subjects had a normal left ventricular filling profile. Of the 43 study patients, 8 (19%) showed Doppler echocardiographic signs of impaired left ventricular relaxation, including a prolonged left ventricular isovolumic relaxation time, decreased peak left ventricular diastolic inflow velocity, and a prolonged mean deceleration time of early diastolic flow velocity. Of these 8 patients, 5 were from Group 1, 1 was from Group 2, and 2 were from Group 3. A positive correlation (r = 0.66; standard error of the estimate = 0.32) was found between the late-to-early left ventricular diastolic inflow velocity ratio and the pulmonary-to-systemic vascular resistance ratio. Our results showed impaired left ventricular relaxation in 8 (19%) of patients with atrial septal defect; 5 of these patients had severe pulmonary hypertension. We therefore conclude that left ventricular diastolic dysfunction is closely related to severe pulmonary hypertension.     

Cyanosis in patients with atrial septal defect due to systemic venous drainage into the left atrium

Cyanosis and clubbing of the digits in patients with atrial septal defect usually are indicative of reversal of the interatrial shunt consequent to reduced right ventricular compliance and severe pulmonary hypertension. The condition may be considered inoperable. The cases reported herein emphasize the occurrence of cyanosis in patients with interatrial communication and continued significant left to right shunting, as a consequence of systemic venoarterial shunting into the left atrium. Carefully planned cardiac catherization is indicated to detect the less well recognized mechanisms of cyanosis so that such patients are not denied the benefits of surgical closure of the septal defect.     

Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. A prospective follow-up study of 21-33 years.

AIMS: Although studies have suggested good long-term results, arrhythmias, pulmonary hypertension and left ventricular dysfunction are mentioned as sequelae long-term after surgical atrial septal defect closure at young age. Most studies were performed only by questionnaire and in a retrospective manner. The long-term outcome is very important with regard to future employment and acceptance on insurance schemes. METHODS AND RESULTS: One hundred and thirty-five consecutive ASD-patients, operated on in childhood, were studied longitudinally with ECG, echocardiography, exercise testing and Holter-recording 15 (10-22) and 26 (21-33) years after surgery. During follow-up no cardiovascular mortality, stroke, heart failure and no pulmonary hypertension occurred. Symptomatic supraventricular tachyarrhythmias were present in 6% after 15 years, and an additional 2% occurred in the last decade; 5% needed pacemaker implantation. No relation was found between arrhythmias and type of ASD, baseline data, right ventricular dimensions, or age at operation. Left and right ventricular function and dimension remained unchanged. Slightly more patients had right atrial dilatation at last follow-up. Exercise capacity was comparable with the normal Dutch population. CONCLUSIONS: The long-term outcome after ASD closure at young age shows excellent survival and low morbidity. The incidence of supraventricular arrhythmias is lower than in natural history studies of ASD patients and also lower than after surgical correction at adult age.     

The right heart in congenital heart disease

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.     

Long-term vardenafil therapy improves hemodynamics in patients with pulmonary hypertension.

The phosphodiesterase-5 (PDE-5) inhibitor, sildenafil, has been reported to produce sustained pulmonary vasodilatation in patients with pulmonary hypertension (PH). Recently, vardenafil, a more potent and selective PDE-5 inhibitor than sildenafil, has been approved for the treatment of erectile dysfunction. However, the long-term effects of oral vardenafil in patients with PH are unknown. We studied five consecutive patients with PH; one with primary pulmonary hypertension, two with chronic pulmonary thromboembolism, one with Eisenmenger syndrome (ventricular septal defect) and one with secondary pulmonary hypertension after a ventricular septal defect closure operation. In an acute hemodynamic trial, vardenafil (5 mg) significantly decreased both the pulmonary vascular resistance (PVR) and systemic vascular resistance (SVR) with an increase in cardiac output. In a chronic hemodynamic trial, the maintenance dose of vardenafil (10 to 15 mg) for 3 months significantly decreased the PVR, but not the SVR, with a 20.7% reduction of the PVR/ SVR ratio. Plasma brain natriuretic peptide (BNP) levels were also significantly decreased after 3 months. This pilot study demonstrates that long-term oral vardenafil therapy may be a safe and effective treatment for patients with PH.     

Left ventricular filling characteristics in pulmonary hypertension: a new mode of ventricular interaction.

OBJECTIVE--To examine the effects of pulmonary hypertension on left ventricular diastolic function and to relate the findings to possible mechanisms of interdependence between the right and left sides of the heart in ventricular disease. DESIGN--A retrospective and prospective analysis of echocardiographic and Doppler studies. SETTING--A tertiary referral centre for both cardiac and pulmonary disease. PATIENTS--29 patients with pulmonary hypertension (12 primary pulmonary hypertension, 10 pulmonary fibrosis, five atrial septal defect (ASD), and two scleroderma) were compared with a control group of 10 patients with an enlarged right ventricle but normal pulmonary artery pressure (six ASD, one after ASD closure, one ASD and pulmonary valvotomy, one tricuspid valve endocarditis and repair, and one pulmonary fibrosis). None had clinical or echocardiographic evidence of intrinsic left ventricular disease. MAIN OUTCOME MEASURES--M mode echocardiographic measurements were made of septal thickness, and left and right ventricular internal cavity dimensions. Doppler derived right ventricular to right atrial pressure drop, and time intervals were measured, as were isovolumic relaxation time, and Doppler left ventricular filling characteristics. RESULTS--The peak right ventricular to right atrial pressure gradient was (mean (SD)) 60 (16) mm Hg in pulmonary hypertensive patients, and 18 (5) mm Hg in controls. The time intervals P2 to the end of the tricuspid regurgitation, and P2 to the start of tricuspid flow were both prolonged in patients with pulmonary hypertension compared with controls (115 (60) and 120 (40) v 40 (15) and 45 (10) ms, p values less than 0.001). Pulmonary hypertensive patients commonly had a dominant A wave on the transmitral Doppler (23/29); however, all the controls had a dominant E wave. Isovolumic relaxation time of the left ventricle was prolonged in pulmonary hypertensive patients compared with controls, measured as both A2 to mitral valve opening (80 (25) v 50 (15) ms) and as A2 to the start of mitral flow (105 (30) v 60 (15) ms, p values less than 0.001). The delay from mitral valve opening to the start of transmitral flow was longer in patients with pulmonary hypertension (30 (15) ms) compared with controls (10 (10) ms, p less than 0.001). At the time of mitral opening there was a right ventricular to right atrial gradient of 12 (10) mm Hg in pulmonary hypertensive patients, but this was negligible in controls (0.4 (0.3) mm Hg, p less than 0.001). CONCLUSIONS--Prolonged decline of right ventricular tension, the direct result of severe pulmonary hypertension, may appear as prolonged tricuspid regurgitation. It persists until after mitral valve opening on the left side of the heart, where events during isovolumic relaxation are disorganised, and subsequent filling is impaired. These effects are likely to be mediated through the interventricular septum, and this right-left ventricular asynchrony may represent a hitherto unrecognised mode of ventricular interaction.     

A case of secundum atrial septal defect combined with aortic stenosis and coronary artery disease showing a single second heart sound

We describe an extremely rare case of secundum atrial septal defect with aortic stenosis and coronary artery disease showing a single second heart sound throughout the respiratory cycle by echocardiogram with simultaneous phonocardiogram. Aortic valve closure corresponded to the single second heart sound. We were unable to detect pulmonary valve closure (PVC) on echocardiogram. Because of the presence of pulmonary hypertension, the pulmonary component of the second heart sound (P2) was presumed to be increased in intensity, and the PVC-P2 interval was thought to be abbreviated. Carotid pulse tracing showed a prolongation of the left ventricular ejection time. We concluded that the single second heart sound was due to both prolongation of left ventricular systole and pulmonary hypertension.     

Clinical characteristics and diagnosis of double-orifice left atrioventricular valve associated with other congenital heart disease

Four cases of double-orifice left atrioventricular (AV) valve are reported. Three of the four patients with double-orifice left AV valve had other associated congenital heart diseases, and the fourth had double-orifice left AV valve alone. A patient with associated ventricular septal defect, who presented with pulmonary congestion and hypertension, suffered from severe heart failure. However, these symptoms improved with the spontaneous closure of the ventricular septal defect. The patient with double-orifice left AV valve alone showed no cardiac symptoms. Two-dimensional and Doppler echocardiography did not detect the double-orifice left AV valve in two of the four patients. More recently, the echocardiographic technique has allowed a noninvasive and more frequent detection of this abnormality. But both of these patients had left-to-right atrial shunt and subsequent reduced transmitral flow and left ventricular volume, which may have made it difficult to detect the morphological and hemodynamic characteristics of double-orifice left AV valve. Careful and repeated echocardiographic observation of mitral configuration is required to determine the presence of double-orifice left AV valve when heart disease is associated with a left-to-right atrial shunt.     

Real-time evaluation of the hemodynamic effects of atrial septal defect closure in adults with left ventricular dysfunction.

Transcatheter closure of atrial septal defects with left-to-right shunt induces an abrupt overload of the left ventricle that may cause acute heart failure in patients with left ventricular dysfunction. We report two cases of ostium secundum atrial septal defects associated with left ventricular dysfunction of different etiology. The hemodynamic evaluation of left ventricular function during transient abolition of the shunt with the sizing balloon of the Amplatzer system helped to establish the most correct therapeutic strategy.     

Acute left ventricular failure after transcatheter closure of a secundum atrial septal defect in a patient with coronary artery disease: a critical reappraisal.

We report a case of acute left ventricular failure after transcatheter closure of a single secundum atrial septal defect in a 68-year-old man with coronary artery disease. Just before the procedure, two coronary lesions had been treated with direct stenting. Transcatheter closure of atrial septal defects should always be deferred in ischemic heart disease patients who need percutaneous myocardial revascularization.     

Left ventricular failure produces profound lung remodeling and pulmonary hypertension in mice: heart failure causes severe lung disease

Chronic left ventricular failure causes pulmonary congestion with increased lung weight and type 2 pulmonary hypertension. Understanding the molecular mechanisms for type 2 pulmonary hypertension and the development of novel treatments for this condition requires a robust experimental animal model and a good understanding of the nature of the resultant pulmonary remodeling. Here we demonstrate that chronic transverse aortic constriction causes massive pulmonary fibrosis and remodeling, as well as type 2 pulmonary hypertension, in mice. Thus, aortic constriction-induced left ventricular dysfunction and increased left ventricular end-diastolic pressure are associated with a ≤5.3-fold increase in lung wet weight and dry weight, pulmonary hypertension, and right ventricular hypertrophy. Interestingly, the aortic constriction-induced increase in lung weight was not associated with pulmonary edema but resulted from profound pulmonary remodeling with a dramatic increase in the percentage of fully muscularized lung vessels, marked vascular and lung fibrosis, myofibroblast proliferation, and leukocyte infiltration. The aortic constriction-induced left ventricular dysfunction was also associated with right ventricular hypertrophy, increased right ventricular end-diastolic pressure, and right atrial hypertrophy. The massive lung fibrosis, leukocyte infiltration, and pulmonary hypertension in mice after transverse aortic constriction clearly indicate that congestive heart failure also causes severe lung disease. The lung fibrosis and leukocyte infiltration may be important mechanisms in the poor clinical outcome in patients with end-stage heart failure. Thus, the effective treatment of left ventricular failure may require additional efforts to reduce lung fibrosis and the inflammatory response.     

Transcatheter Closure of Right‐to‐Left Interatrial Shunts to Resolve Hypoxemia

Background. Right‐to‐left interatrial shunts through an atrial septal defect or patent foramen ovale may result from congenital and acquired conditions. Right‐to‐left shunts may occur with normal pulmonary artery pressure and resistance as in right ventricular outflow tract obstruction leading to hypertrophy and reduced diastolic function, in right ventricle ischemia, myocarditis or cardiomyopathy and in orthodeoxia–platypnea syndrome. Methods. We have tested the tolerance of trans‐catheter closure of right‐to‐left interatrial shunts in 5 selected patients in whom the morphology and hemodynamics implied that it would be well‐tolerated and improve functional capacity. Right‐to‐left shunt was demonstrated in 5 profoundly cyanotic patients by trans‐thoracic and trans‐esophageal echocardiography with intravenous injection of agitated saline. The patients were catheterized for hemo‐dynamic study and tested for tolerance of transient balloon occlusion of the defects. Results. Diastolic right ventricular dysfunction with elevated end‐diastolic pressure was the primary cause of right‐to‐left shunt. Most shunts occur via atrial septal defects. Patients' ability to tolerate temporary balloon occlusion of the defects predicted a favorable outcome following permanent device occlusion. Cyanosis resolved in all patients following closure of the defects without congestive right heart failure. A marked improvement in functional capacity was observed in 4 patients. One died of preinterventional hypoxic brain damage. Conclusion. Transcatheter closure of right‐to‐left shunts is well‐tolerated and a rewarding approach. It should be applied in selected patients following careful morphologic and hemodynamic evaluation. Tolerance of temporary occlusion of the defect is predictive of a favorable procedural outcome.     

Percutaneous closure of an atrial septal defect in an infant with shone's syndrome

Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow‐up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures. © 2012 Wiley Periodicals, Inc.     

Pulmonary hypertension and right ventricular dysfunction in left heart disease (group 2 pulmonary hypertension)

Group 2 pulmonary hypertension is most frequently caused by left heart disease, a heterogeneous set of disorders. These processes include left ventricular systolic dysfunction, left ventricular dysfunction with preserved ejection fraction and valvular (mitral and/or aortic) diseases. Left heart disease may cause passive backward transmission of pressure leading to elevated left atrial and pulmonary arterial pressures due to a myriad of processes. Increasingly, it has been recognized that some patients may develop pulmonary arterial pressure out of proportion from what is expected. This is believed to be due to increases in vasomotor tone and/or vascular remodeling. Over time patients may go on to develop progressive right ventricular dysfunction, a marker for poor prognosis. This review will explore the different characteristics of these conditions including the incidence, pathophysiology, clinical implications, prognosis and current state of available medical therapies.     

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%.     

Fenestrated ASD Closure in a Child with Idiopathic Pulmonary Hypertension and Exercise Desaturation

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right‐to‐left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½‐year‐old was diagnosed with pulmonary hypertension, a large atrial septal defect and right‐to‐left shunting. Medical therapy over an 18‐month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left‐to‐right atrial flow.