单纯性单侧肺动脉缺如的临床特点及回顾性分析

目的:提高对单纯性单侧肺动脉缺如(UAPA)的认识和早期诊治水平。方法:结合我院2009年经肺血管造影检查明确诊断的1例单纯性单侧肺动脉缺如的临床资料,和国内1999年至2009年文献检索到的13例单纯性单侧肺动脉缺如病例的详细资料,对UAPA的临床特点进行回顾性总结分析。结果:我院患者以胸闷憋气症状就诊,曾于外院误诊为"肺栓塞",行多排CT肺动脉造影(CTPA)、X线肺血管造影(CPA)最终确诊单纯性UAPA。总结14例病例后发现单纯性UAPA病例以成年人多见(10/14),女性8例,男性6例,左侧缺如6例,右侧缺如8例,主要症状为:咳嗽、咳痰、反复肺部感染(9/14),活动后胸闷、气促、喘息(9/14),咯血(3/14),最终以选择性肺血管造影检查和多排螺旋CT肺血管造影确诊病例为最多,分别为6例,5例。在8例有治疗资料中,对症治疗4例(4/14),手术治疗4例(4/14)。结论:UAPA是一种罕见的先天性肺血管畸形,单纯性UAPA更为少见,症状以咳嗽、咳痰、咯血、反复肺部感染、胸闷及气促为主。目前X线肺血管造影仍然是诊断UAPA的金标准。近几年多排螺旋CT肺血管造影、核磁共振(MRI)肺血管造影正逐渐成为诊断UAPA的重要手段之一。目前单纯UAPA的治疗方法主要以对症治疗为主,外科治疗可根据病情选择患侧肺动脉重建、选择性肺侧支血管栓塞和结扎、患侧全肺或肺叶切除术。     

Pulmonary thromboembolism angiographically confirmed: clinical and prognostic aspects]

OBJECTIVE: The aim of this study was to evaluate the clinical profile and prognosis of patients with an angiographically proven thromboembolism. METHODS: Data from 22 consecutive patients (13 males, 9 females; mean age 57.3 +/- 16.8 years) with pulmonary embolism confirmed by pulmonary angiography were reviewed. All our patients were previously submitted to non-invasive diagnostic procedures (blood examinations, EKG, chest x-ray, echocardiography). A V/Q scan was also performed in 5 patients. Fourteen patients were traditionally treated with heparin alone and the rest received thrombolytic therapy and heparin. Two patients had a thromboembolectomy. At discharge, all our patients were submitted to an oral anticoagulant therapy. The mean duration of the follow-up period was 26 +/- 12 months. RESULTS: The majority of the patients were in the 6th decade of life and it was possible to identify a hypercoagulable state in 82%. The most common symptom at the time of presentation was sudden chest pain (64%). The most specific sign in non-invasive procedures were right side cardiac dilatation seen echocardiographically (73%) and the mismatch in the V/Q scan (80%). The most common haemodynamic parameter (91%) observed in the right heart catheterization of these patients was the finding of a gradient between diastolic pulmonary artery and pulmonary capillary wedge pressures. Uneventful angiography was performed in all patients who showed massive pulmonary embolism (86%). Three patients (13.6%) died during the acute phase. At the end of the follow-up period, 10 patients were asymptomatic and 5 had heart failure. Four died, which corresponds to an overall mortality of 31.8% in 2.2 years of follow-up. None of the clinical or haemodynamic parameters analyzed (age, gender, arterial blood gases at presentation, hypercoagulable states, thrombolysis, pulmonary hypertension and extension of the embolism) were related to mortality. CONCLUSION: Angiographically confirmed pulmonary thromboembolism is still a poor outcome situation, even when a lot of diagnostic and therapeutic procedures are available.     

Pulmonary involvement in systemic scleroderma. Part II. Isolated pulmonary arterial hypertension, bronchopulmonary cancer, alveolar hemorrhage

INTRODUCTION: Pulmonary interstitial fibrosis is the most frequent cause of lung disease in systemic sclerosis. However, other pulmonary complications exist, including lung cancer, alveolar hemorrhage, and in particular isolated pulmonary arterial hypertension, which is still considered the bête noire as regards this disease. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of pulmonary arterial hypertension has been reported to range from 5 to 60% in cases of systemic sclerosis; isolated pulmonary arterial hypertension has been principally observed in subjects with a ten-year history of limited forms of the disease. As the patient remains asymptomatic for a long period, with nonspecific respiratory clinical manifestations, the diagnosis is made at a much later stage in the course of the disease. The diagnostic method of choice is echocardiography-doppler, which should be performed during the preliminary investigation, and at follow-up. The prognosis is poor, and patient survival rate at 2 years after onset of symptoms amounts to 40%. To date, no curative therapy for pulmonary arterial hypertension has yet been found. FUTURE PROSPECTS AND PROJECTS: A knowledge of the mechanisms involved in the development of isolated pulmonary arterial hypertension is essential to the determination of new and relevant therapeutic strategies. Vasodilatory treatment, notably calcium channel blockers, prostacyclin and analogs such as iloprost, may be effective at an early stage of the disease before the appearance of permanent vascular damage.     

Early and late results of the modified Waterston shunt with PTFE grafts for palliation of complex congenital cyanotic heart disease in neonates.

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)     

Complications of cardiac catheterization in the current era: a single-center experience.

Consecutive cardiac catheterization procedures done over a 2-yr period (April 1996 to March 1998) were prospectively analyzed to determine and characterize procedure-related complications (in-hospital and 1-mo follow-up), as they occur at present. During the study period, 11,821 procedures (7,953 diagnostic and 3,868 therapeutic) were performed. The majority of procedures (> 60%) were done in high-risk patients. Stents were implanted in 33% of patients, and adjunctive abciximab was used in 6.6% of therapeutic procedures. The overall complication rate was 8% (3.6% of diagnostic procedures and 15.1% of therapeutic procedures). The procedure-related mortality rates were 0.2%, 0.1%, and 0.5% for total, diagnostic, and therapeutic procedures, respectively. Cardiac complications were seen in 3.9% (1.5% of diagnostic and 9% of therapeutic procedures). Emergency cardiac surgery was required in 0.05% of the diagnostic procedure group and 0.3% of the therapeutic procedure group (total, 0.1%). Despite marked changes in patient population and practice, the complication rates of cardiac catheterization remain very low.     

Pulmonary hypertension screening in systemic scleroderma: a cohort study of 67 patients

PURPOSE: Pulmonary hypertension is a severe complication of systemic sclerosis and has emerged as a major cause of morbidity and mortality in this condition. Treatment is all the more efficient as pulmonary hypertension is early diagnosed. A good knowledge of the clinical, biological and functional features of pulmonary hypertension in systemic sclerosis is therefore necessary to suspect and to diagnose pulmonary hypertension as early as possible. METHODS: Sixty seven patients with systemic sclerosis were retrospectively studied. We compared clinical, immunological, functional (spirometry) and morphological (pulmonary fibrosis) features according to the presence (n = 25) and the characteristic of pulmonary hypertension (isolated or secondary) or the absence (n = 42) of pulmonary hypertension, assessed by Doppler echocardiography. RESULTS: CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly and telangiectasia) was more frequent in patients with isolated pulmonary hypertension than in patients without PH (72.7% vs 28.5%, P < 0.05; odds-ratio [OR] = 6.6) and dyspnea was more severe (P < 0.001; OR = 11.4). The age at time of pulmonary hypertension diagnosis was higher in patients with secondary pulmonary hypertension than in patients with isolated from (median: 62.5 years (range: 32-35) vs 53 years (range: 37-85), P < 0.05). Patients with isolated pulmonary hypertension had anticardiolipin antibodies more frequently than patients without pulmonary hypertension (72.7% vs 35.7%, P < 0.05). Isolated reduction of diffusing capacity was preferentially observed among patients with isolated pulmonary hypertension than among those without pulmonary hypertension. A linear relation between systolic pulmonary artery pressure values and diffusing capacity values (r = 0.72, P < 0.01) was found. Isolated reduction of diffusing capacity was more frequent in patients with isolated pulmonary hypertension than in patients without pulmonary hypertension (63.6% vs 14.3%, P < 0.001; OR = 10.5). CONCLUSION: The severity of pulmonary hypertension in systemic sclerosis justifies a systematic screening by Doppler echocardiography and diffusing capacity measurement. Our results allow us to better define the characteristics of sclerodermic patients with isolated or secondary pulmonary hypertension. The search for pulmonary hypertension should be repeated with time and clinicians should be particularly vigilant in the case of a patient presenting these characteristics.     

Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers.

OBJECTIVES: This study sought to assess the risks associated with right heart catheter procedures in patients with pulmonary hypertension. BACKGROUND: Right heart catheterization, pulmonary vasoreactivity testing, and pulmonary angiography are established diagnostic tools in patients with pulmonary hypertension, but the risks associated with these procedures have not been systematically evaluated in a multicenter study. METHODS: We performed a multicenter 5-year retrospective and 6-month prospective evaluation of serious adverse events related to right heart catheter procedures in patients with pulmonary hypertension, as defined by a mean pulmonary artery pressure >25 mm Hg at rest, undergoing right heart catheterization with or without pulmonary vasoreactivity testing or pulmonary angiography. RESULTS: During the retrospective period, 5,727 right heart catheter procedures were reported, and 1,491 were reported from the prospective period, for a total of 7,218 right heart catheter procedures performed. The results from the retrospective and the prospective analyses were almost identical. The overall number of serious adverse events was 76 (1.1%, 95% confidence interval 0.8% to 1.3%). The most frequent complications were related to venous access (e.g., hematoma, pneumothorax), followed by arrhythmias and hypotensive episodes related to vagal reactions or pulmonary vasoreactivity testing. The vast majority of these complications were mild to moderate in intensity and resolved either spontaneously or after appropriate intervention. Four fatal events were recorded in association with any of the catheter procedures, resulting in an overall procedure-related mortality of 0.055% (95% confidence interval 0.01% to 0.099%). CONCLUSIONS: When performed in experienced centers, right heart catheter procedures in patients with pulmonary hypertension are associated with low morbidity and mortality rates.     

先天性单侧肺动脉缺如25例影像学诊断

目的:评价先天性一侧肺动脉缺如(UAPA)多排螺旋CTA的影像学诊断价值。方法:回顾分析25例我院明确诊断为先天性一侧肺动脉缺如的胸X线片、经胸超声心动图检查、胸部多排螺旋CTA及心血管造影表现。结果:15(15/25)例为右肺动脉缺如,10(10/25)例为左肺动脉缺如。单发UAPA 4例,均为右肺动脉缺如。UAPA合并其他心血管畸形21例,其中合并简单心血管畸形9例,合并复杂畸形12例,简单畸形中包括动脉导管未闭、房间隔缺损、室间隔缺损等,复杂畸形中包括肺动脉闭锁、法洛四联症各4例,肺动脉狭窄3例,另有大动脉转位等。9例经外科手术证实,12例经心血管造影证实。结论:对于一侧肺动脉缺如的诊断,多排螺旋CTA检查作为一种无创的检查方法与心血管造影同样具有明确诊断价值,CTA所显示的客观的解剖结构,为治疗方案的选择提供依据。     

Congenital unilateral absence of a pulmonary artery: a report of two adult cases

Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly that is generally diagnosed in childhood and frequently associated with other cardiovascular abnormalities. Two patients with unilateral absence of a pulmonary artery first diagnosed as adults are reported. Our two cases demonstrate that presentation of UAPA may range from an incidental finding to chronic, life-threatening symptoms. Because UAPA may require urgent intervention, physicians should be alerted to the possibility of this entity presenting later in life.     

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.     

Long-term surgical prognosis of aortic valve diseases with pulmonary hypertension. Apropos of 34 cases]

Thirty-four patients underwent isolated aortic valve replacement with mean pulmonary artery pressures greater than 40 mmHg between 1972 and 1988. The aortic valve disease was stenotic in 10 cases, regurgitant in 14 cases and mixed in 10 cases. Thirty patients (88%) had invalidating cardiac failure (NYHA Classes III and IV). The mean preoperative ejection fraction was 44 +/- 15%. The hospital mortality was 17.6%. Ten patients died secondarily, five with terminal cardiac failure. The 5 year actuarial survival was 70 +/- 16%; the 10 year survival was 60 +/- 18% with an average follow-up of 115 +/- 61 months. None of the patients was lost to follow-up. Fifteen of the 18 survivors (83%) are asymptomatic or pauci-symptomatic after a follow-up of 126 +/- 62 months. Doppler echocardiography (n = 12) showed normal prosthetic valve function in 11 cases and aortic regurgitation in 1 case. Eight patients had tricuspid regurgitation with pulmonary artery systolic pressures less than 30 mmHg in 6 cases and between 30 and 40 mmHg in 2 cases. Severe pulmonary hypertension is therefore a poor early postoperative prognostic factor in aortic valve replacement surgery due to the associated left ventricular dysfunction. However, the long-term results are satisfactory: clinical improvement is usually related to a reduction of pulmonary hypertension.     

Outcomes of Percutaneous Closure of Patent Ductus Arteriosus Accompanied With Unilateral Absence of Pulmonary Artery

Background

Limited data have reported the outcomes of percutaneous closure of patent ductus arteriosus (PDA) in patients with unilateral absence of pulmonary artery (UAPA). This study aimed to evaluate the symptomatology, diagnosis and therapy, especially the transcatheter closure of PDA in patients with PDA associated with UAPA.

SpyGlass single-operator peroral cholangiopancreatoscopy system for the diagnosis and therapy of bile-duct disorders: a clinical feasibility study (with video)

BACKGROUND: Clinical implementation of cholangioscopy for direct visual examination of bile ducts, tissue sampling, and therapeutic maneuvers has been slowed by limitations in available technology. With 4-way deflected steering and dedicated irrigation channels, the single-operator SpyGlass peroral cholangiopancreatoscopy system is designed to overcome some of these limitations. OBJECTIVE: To evaluate the clinical utility and safety of the SpyGlass system for diagnostic and therapeutic endoscopic procedures in bile ducts. DESIGN: Prospective observational clinical feasibility study. SETTING: Two tertiary referral centers. MAIN OUTCOME MEASUREMENTS: Procedural success rate defined as the proportion of SpyGlass procedures in which the diagnostic or therapeutic objectives of the procedure were achieved. RESULTS: SpyGlass procedures were performed in 35 patients: 22 with indeterminate strictures (63%), 5 with indeterminate filling defects (14%), 5 with stones (14%), 2 with cystic lesions (6%), and 1 patient with an indication for gallbladder stent placement (3%). The rate of procedural success was 91% (95% confidence interval 77%-98%). Twenty patients underwent SpyGlass-directed biopsy, and the specimens procured from 19 patients (95%) were found adequate for histologic evaluation. The preliminary sensitivity and specificity of SpyGlass-directed biopsy to diagnose malignancy were 71% and 100%, respectively. SpyGlass-directed electrohydraulic lithotripsy succeeded in 5 of 5 patients (100%). Procedure-related complications occurred in 2 patients (6%) and resolved uneventfully. LIMITATIONS: No control group was included. Follow-up for determining preliminary sensitivity and specificity was limited. CONCLUSIONS: SpyGlass procedures proved to be clinically feasible, provided adequate samples for histologic diagnosis, and successfully guided stone therapy. The procedures were safe and well tolerated. Prospective multicenter clinical trials of the system are underway.     

Primary acute pericardial disease: a prospective series of 231 consecutive patients

A series of 231 patients with "primary" acute pericardial disease (acute pericarditis or tamponade presenting without an apparent cause) were studied according to the following protocol: general clinical and laboratory studies (stage I), pericardiocentesis (stage II), pericardial biopsy (stage III) and blind antituberculous therapy (stage IV). In 32 patients (14%) a specific etiologic diagnosis was obtained (13 with neoplasia, 9 with tuberculosis, 4 with collagen vascular disease, 2 with toxoplasmosis, 2 with purulent pericarditis and 2 with viral pericarditis). "Diagnostic" pericardiocentesis (32 patients) was performed when clinical activity and effusion persisted for longer than 1 week or when purulent pericarditis was suspected, whereas "therapeutic" pericardiocentesis (44 patients) was performed to treat tamponade; their diagnostic yield was 6% and 29%, respectively. "Diagnostic" biopsy (20 patients) was carried out when illness persisted for longer than 3 weeks, whereas "therapeutic" biopsy was performed whenever pericardiocentesis failed to relieve tamponade; their diagnostic yield was 5% and 54%, respectively. The diagnostic yield difference between "diagnostic" and "therapeutic" procedures was significant (p less than 0.001); in contrast, the global diagnostic yield of pericardiocentesis (19%) and biopsy (22%) was similar. At the end of follow-up (1 to 76 months, mean 31 +/- 20), no patient in whom a diagnosis of idiopathic pericarditis had been made showed signs of pericardial disease. It is concluded that a "diagnostic" procedure is not warranted as a routine method, a choice between "therapeutic" pericardiocentesis and biopsy is circumstantial and must be individualized, and only through a systematic approach can a substantial diagnostic yield be reached in primary acute pericardial disease.     

Helical computerized tomography of the thorax in the diagnosis of unresolved chronic pulmonary thromboembolism

We assessed the diagnostic usefulness of helical CT scan of the thorax in the setting of chronic thromboembolic pulmonary hypertension by prospectively comparing the results of helical CT scan to those of the pulmonary angiogram (gold standard). We studied 40 patients with diagnosis of pulmonary hypertension of diverse etiology (mean age: 40.7 +/- 12 y.o.; mean systolic pulmonary artery pressure: 91 +/- 33 mmHg)). Thirty of these patients fulfilled the diagnostic criteria of chronic thromboembolic pulmonary hypertension and the other ten were used as controls. Diagnosis in control patients included: primary pulmonary hypertension (4); patent ductus arteriosus (2); atrial septal defect (1); rheumatic valve disease (1); ischemic heart disease (1); and acute pulmonary embolism (1). Both helical CT scan and pulmonary angiogram were part of the routine diagnostic work up of these patients, and were, performed and interpreted almost simultaneously (within one week) by a different group of investigators in a blind manner. Only the diagnostic accuracy of the method regarding central (major arteries) vascular lesions was evaluated. Helical CT scan had an overall sensitivity of 100% (29/29), and a specificity of 91% (10/11). Positive predictive and negative predictive values were 96.6% (29/30) and 100% (10/10), respectively. Overall diagnostic accuracy was 97.5% (39/40). We conclude that helical CT scan of the thorax is an excellent alternative approach for the diagnosis of major arteries lesions in the setting of chronic thromboembolic pulmonary hypertension.     

Coccidioidomycosis: a descriptive survey of a reemerging disease. Clinical characteristics and current controversies

Coccidioidomycosis is a fungal disease with protean manifestations endemic to the Lower Sonoran Life Zone, which includes the hot deserts of the southwestern United States and areas of Mexico. Two hundred and twenty-three patients were found to have coccidioidomycosis at our institution from 1994-2002, the largest reported cohort of coccidioidomycosis patients since the 1950s. Of these patients, 58% presented with isolated pulmonary disease, 14% had high (>1:16) complement fixation titers without clear evidence of dissemination, 22% had definite disseminated disease, and 5% had unclassified disease. Enzyme immunoassay was a reliable diagnostic tool in those with symptomatic disease, but had a low specificity in those who were asymptomatic. Complement fixation titers of > or =1:16 were associated with dissemination to bone or skin but were not helpful in evaluating central nervous system disease. Thirteen percent of patients with high complement fixation titers (>1:16) without clear evidence of dissemination on presentation and 7% of those with isolated pulmonary disease eventually progressed to disseminated disease; 30% of Filipino patients with pulmonary disease progressed to disseminated disease. Nonwhite race was a predictor for dissemination; African American patients more often developed disseminated bony disease while Filipinos were more likely to develop cutaneous or central nervous system disease. Relapse of disseminated coccidioidomycosis occurred in 24% of patients; the risk was highest (71%) among those with central nervous system disease. Azole therapy was generally inferior to amphotericin B in disseminated disease. Predictors of permanent disability included African American or Filipino race, central nervous system disease, and bony disease.     

Chronic thromboembolic pulmonary hypertension

Over the past 4 decades, chronic thromboembolic pulmonary hypertension has evolved from an autopsy curiosity to a potentially correctable form of pulmonary hypertension. Advances in surgical techniques along with the introduction of pulmonary hypertension disease-modifying therapies provide a therapeutic option for the majority of patients afflicted with the disease. Approximately 5,000 thromboendarterectomy procedures have now been performed worldwide with mortality rates reported by established programs experienced in the management of this disease process falling to a range of 4 to 7%. A mortality rate of 1.3% has been reported in patients at low risk based on their preoperative hemodynamic profile. After a successful pulmonary thromboendarterectomy, substantial improvement and often normalization can be achieved in right ventricular function, gas exchange, exercise capacity, and quality of life. For patients not candidates for thromboendarterectomy, or for those with persistent post-thromboendarterectomy pulmonary hypertension, disease-modifying medical therapies have been demonstrated to stabilize and improve pulmonary hemodynamics, albeit not to the same extent as primary thromboendarterectomy. The current review focuses on the diagnostic approach to chronic thromboembolic pulmonary hypertension and the available surgical and medical therapeutic options. Additional research is necessary to more accurately predict postoperative hemodynamic outcome and to define the optimal therapeutic approach, especially in patients with involvement of the distal vasculature.     

一侧肺动脉缺如合并先天性心脏病的介入治疗分析

目的:总结经导管介入治疗一侧肺动脉缺如合并先天性心脏病的疗效。方法:我科自2005年1月至2009年4月诊断5例一侧肺动脉缺如合并先天性心脏畸形,其中合并动脉导管未闭4例,合并房间隔缺损1例;左肺动脉缺如1例,右肺动脉缺如4例;本组男性1例,女性4例,年龄1.5～11岁,3例肺部反复感染、1例咯血及1例发绀。均通过临床体检、X线胸片、心电图、经胸超声心动图检查及多排CT或造影确诊。结果:4例合并动脉导管通过介入治疗成功封堵。术后平均3d出院,未出现并发症,随诊3个月～4年心脏均恢复正常大小。1例肺动脉高压为极重度,合并房间隔缺损,出现发绀,不能除外原发肺动脉高压因素,放弃介入治疗,予口服扩血管药物治疗,出院随诊。结论:一侧肺动脉缺如合并先天性心脏病临床常表现为中、重度肺动脉高压多伴一侧肺发育不良,手术风险大。应用介入治疗显示出创伤小、并发症少,恢复快等优势,挽救了一些外科也难以处理的高风险重症患儿。     

Right ventricular dilatation and intraventricular septal motion after surgical closure of atrial septal defect

The persistence of right ventricular dilatation and paradoxical interventricular septal motion are two echocardiographic abnormalities rarely reported after surgical closure of atrial septal defects. The aim of this study was to identify the predictive factors of these abnormalities in the long-term and to study their functional consequences. One hundred and two patients aged 18 +/- 14 years (range 1-62 years) underwent closure of atrial septal defects. Thirty-five patients were under 10 years of age, 33 were 10 to 20 years of age and 34 were over 20. Fifty-six patients were female. The rhythm was sinus in the great majority of cases (97%). Three patients, all over 40 years of age, were in atrial fibrillation. Before surgery, right ventricular dilatation was observed in 95 patients (91.2%), paradoxical septal wall motion in 93 patients (91.2%), the ratio of pulmonary/systemic output was 2.7 +/- 0.6 (range 1.7 to 7.4) and over 2 in 90% of patients: pulmonary systolic pressure was 32.3 +/- 12 mmHg and over 40 mmHg in 18 patients (17.6%). Ninety-four patients were followed up regularly with a mean follow-up time of 5.5 +/- 3.6 years (1-14 years). The right ventricle remained dilated in 37 patients (39.4%) after surgery: the right ventricular dimension decreased from 36 +/- 1 to 27.8 +/- 6.2 mm (p = 0.001). The ratio of end diastolic right ventricular/left ventricular dimension also decreased from 1.07 +/- 0.31 to 0.56 +/- 0.12 (p = 0.0001). Multivariate analysis identified two predictive factors of persistent right ventricular dilatation: age > 40 years (p = 0.009) and a pulmonary/systemic flow ratio > 3 (p = 0.03). Interventricular septal wall motion remained paradoxical in 21 patients (22%). Multivariate analysis identified two predictive factors of persistent paradoxical septal motion: age > 40 years (p = 0.02) and systolic pulmonary pressures > 40 mmHg (p = 0.03). These abnormalities remained asymptomatic in all but two patients with persistent long-term hypertension and a residual atrial septal defect. The persistence of right ventricular dilatation and paradoxical septal motion was quite common, with older age at surgery, systolic pulmonary artery pressure > 40 mmHg and a ratio of pulmonary/systemic blood flow > 3, being predisposing factors. These abnormalities were clinically asymptomatic when isolated.