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Cardiol J ; 26(5): 451-458, 2019.
Article En | MEDLINE | ID: mdl-30246235

BACKGROUND: Thermal injury during radiofrequency ablation (RFA) of atrial fibrillation (AF) can lead to pulmonary vein stenosis (PVS). It is currently unclear if routine screening for PVS by imaging (echocardiography, computed tomography) is clinically meaningful and if there is a correlation between PVS and the electroanatomical mapping system (EAMS) used for the ablation procedure. It was therefore investigated in the current single center experience. METHODS: All patients from January 2004 to December 2016 with the diagnosis of PVS after interventional ablation of AF by radiofrequency were retrospectively analyzed. From 2004 to 2007, transesophageal echocardiography was routinely performed as screening for RFA-acquired PVS (group A). Since 2008, diagnostics were only initiated in cases of clinical symptoms suggestive for PVS (group B). RESULTS: The overall PVS rate after interventional RFA for AF of the documented institution is 0.72% (70/9754). The incidence was not influenced by screening: group A had a 0.74% PVS rate and group B a 0.72% rate (NS). Referred to as the EAMS, there were significant differences: 20/4229 (0.5%) using CARTO®, 48/4510 (1.1%) using EnSite®, 1/853 (0.1%) using MediGuide®, and 1/162 (0.6%) using Rhythmia®. Since 2009, no significant difference between technologies was found. CONCLUSIONS: The present analysis of 9754 procedures revealed 70 cases of PVS. The incidence of PVS is not related to screening but to the application of different EAMS. Possible explanations are technological backgrounds (magnetic vs. electrical), learning curves, operator experience, and work-flow differences. Furthermore, incorporation of new technologies seems to be associated with higher incidences of PVS before workflows are optimized.

Atrial Fibrillation/therapy , Catheter Ablation/adverse effects , Electrophysiologic Techniques, Cardiac/adverse effects , Pulmonary Veins/surgery , Pulmonary Veno-Occlusive Disease/epidemiology , Vascular System Injuries/etiology , Aged , Atrial Fibrillation/diagnosis , Atrial Fibrillation/epidemiology , Atrial Fibrillation/physiopathology , Clinical Competence , Echocardiography, Transesophageal , Female , Germany/epidemiology , Humans , Incidence , Learning Curve , Male , Middle Aged , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/injuries , Pulmonary Veins/physiopathology , Pulmonary Veno-Occlusive Disease/diagnostic imaging , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome , Vascular System Injuries/diagnostic imaging , Workflow
Int J Clin Pharm ; 40(4): 790-794, 2018 Aug.
Article En | MEDLINE | ID: mdl-30101375

Spontaneous reporting is the primary method used in pharmacovigilance (PV) to detect drug safety signal. Specific criteria used in pharmacovigilance to prove accountability of a drug are rarely present in rare disease. The low number of alerts also makes it challenging. The aim of this commentary is to raise awareness among pharmacists on issues and opportunities for pharmacovigilance in rare diseases, taking pulmonary arterial hypertension (PAH) as example, from which a subset of cases are drug-induced. It is demonstrated how a dedicated program named VIGIAPATH created to reinforce pharmacovigilance of drug-induced pulmonary arterial hypertension at a national level, led to increase self-reporting and confirm safety signals. Thanks to a specific program such as VIGIAPATH, pharmacists can play an important role in communication with clinicians, patients and regulatory agencies, facilitating the detection of potential safety signals at an early stage in rare disease.

Adverse Drug Reaction Reporting Systems , Antibiotics, Antineoplastic/adverse effects , Hypertension, Pulmonary/chemically induced , Mitomycin/adverse effects , Pharmacovigilance , Protein Kinase Inhibitors/adverse effects , Pulmonary Veno-Occlusive Disease/chemically induced , Attitude of Health Personnel , Diagnosis, Differential , Health Knowledge, Attitudes, Practice , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/physiopathology , Interdisciplinary Communication , Patient Safety , Pharmacists/psychology , Predictive Value of Tests , Professional Role , Program Evaluation , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/physiopathology , Risk Assessment , Risk Factors
Rev Mal Respir ; 35(2): 160-170, 2018 Feb.
Article Fr | MEDLINE | ID: mdl-29501213

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding. A noninvasive diagnostic approach, including oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest, is used to support a diagnosis of PVOD. PVOD prognosis is worse than other forms of PAH. There is no evidence-based medical therapy for PVOD and life-threatening pulmonary edema may occur following PAH targeted therapy in PVOD. Lung transplantation remains the preferred definitive therapy for eligible patients.

Pulmonary Veno-Occlusive Disease , Animals , Diagnostic Imaging/methods , Disease Models, Animal , Humans , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/therapy , Rare Diseases/diagnosis , Rare Diseases/epidemiology , Rare Diseases/therapy , Respiratory Function Tests/methods , Risk Factors
Interact Cardiovasc Thorac Surg ; 27(1): 20-26, 2018 07 01.
Article En | MEDLINE | ID: mdl-29474564

OBJECTIVES: Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity. METHODS: A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach. RESULTS: There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight <2 kg and with postoperative PVO (P < 0.0001, P = 0.0041, P = 0.0003, respectively). Reoperations were performed 27 times in 22 patients (PVO repair, 11; staged repair, 4 and others, 12). PVO repair was performed at a mean of 2.5 ± 1.6 months later. The actuarial freedom from reoperation rates were 88.8% and 83.2% at 20 and 30 years, respectively. Multivariable analysis revealed that the risk of reoperation was associated with mixed-type TAPVC and postoperative PVO (P = 0.0064 and P < 0.0001, respectively). CONCLUSIONS: Long-term surgical outcomes of isolated TAPVC have improved over the past 25 years. Postoperative PVO, the mixed-type TAPVC and a body weight <2 kg might be the important factors contributing to mortality and morbidity.

Postoperative Complications/epidemiology , Pulmonary Veno-Occlusive Disease/epidemiology , Scimitar Syndrome/surgery , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Pulmonary Circulation , Reoperation , Retrospective Studies , Risk Factors , Treatment Outcome , Young Adult
Respir Med ; 134: 47-53, 2018 01.
Article En | MEDLINE | ID: mdl-29413507

Trichloroethylene (TCE) is a chlorinated solvent that has been used widely around the world in the twentieth century for metal degreasing and dry cleaning. Although TCE displays general toxicity and is classified as a human carcinogen, the association between TCE exposure and respiratory disorders are conflicting. In this review we aimed to systematically evaluate the current evidence for the respiratory effects of TCE exposure and the implications for the practicing clinician. There is limited evidence of an increased risk of lung cancer associated with TCE exposure based on animal and human data. However, the effect of other chlorinated solvents and mixed solvent exposure should be further investigated. Limited data are available to support an association between TCE exposure and respiratory tract disorders such as asthma, chronic bronchitis, or rhinitis. The most consistent data is the association of TCE with autoimmune and vascular diseases such as systemic sclerosis and pulmonary veno-occlusive disease. Although recent data are reassuring regarding the absence of an increased lung cancer risk with TCE exposure, clinicians should be aware of other potential respiratory effects of TCE. In particular, occupational exposure to TCE has been linked to less common conditions such as systemic sclerosis and pulmonary veno-occlusive disease.

Occupational Diseases/chemically induced , Respiration Disorders/chemically induced , Solvents/adverse effects , Trichloroethylene/adverse effects , Chronic Disease , Humans , Lung Neoplasms/chemically induced , Lung Neoplasms/epidemiology , Occupational Diseases/epidemiology , Occupational Exposure/adverse effects , Pulmonary Veno-Occlusive Disease/chemically induced , Pulmonary Veno-Occlusive Disease/epidemiology , Respiration Disorders/epidemiology , Solvents/pharmacokinetics , Trichloroethylene/pharmacokinetics
Eur Respir J ; 46(6): 1721-31, 2015 Dec.
Article En | MEDLINE | ID: mdl-26541523

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension characterised by predominant remodelling of pulmonary venules. Bi-allelic mutations in the eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4) gene were recently described as the major cause of heritable PVOD, but risk factors associated with PVOD remain poorly understood. Occupational exposures have been proposed as a potential risk factor for PVOD, but epidemiological studies are lacking.A case-control study was conducted in consecutive PVOD (cases, n=33) and pulmonary arterial hypertension patients (controls, n=65). Occupational exposure was evaluated via questionnaire interview with blinded assessments using an expert consensus approach and a job exposure matrix (JEM).Using the expert consensus approach, PVOD was significantly associated with occupational exposure to organic solvents (adjusted OR 12.8, 95% CI 2.7-60.8), with trichloroethylene being the main agent implicated (adjusted OR 8.2, 95% CI 1.4-49.4). JEM analysis independently confirmed the association between PVOD and trichloroethylene exposure. Absence of significant trichloroethylene exposure was associated with a younger age of disease (54.8±21.4 years, p=0.037) and a high prevalence of harbouring bi-allelic EIF2AK4 mutations (41.7% versus 0%, p=0.015).Occupational exposure to organic solvents may represent a novel risk factor for PVOD. Genetic background and environmental exposure appear to influence the phenotypic expression of the disease.

Occupational Exposure/statistics & numerical data , Protein-Serine-Threonine Kinases/genetics , Pulmonary Veno-Occlusive Disease/epidemiology , Solvents , Trichloroethylene , Adult , Age of Onset , Aged , Aged, 80 and over , Bone Morphogenetic Protein Receptors, Type II/genetics , Case-Control Studies , Female , Gene-Environment Interaction , Genetic Predisposition to Disease , Humans , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/genetics , Logistic Models , Lung/pathology , Male , Middle Aged , Multivariate Analysis , Pulmonary Veno-Occlusive Disease/genetics , Pulmonary Veno-Occlusive Disease/pathology , Risk Factors , Surveys and Questionnaires , Young Adult
Chest ; 146(1): 167-174, 2014 Jul.
Article En | MEDLINE | ID: mdl-24503954

BACKGROUND: Pulmonary venoocclusive disease (PVOD) is a rare lung disease, diagnosed in 5% to 10% of patients with pulmonary hypertension (PH). The incidence, prevalence, and etiology of PVOD in children are not well defined. The mortality remains high, related, at least partly, to the limited treatment options. METHODS: This retrospective analysis (1985-2011) summarizes symptoms, associated factors, treatment, and outcomes of nine pediatric patients (five girls, four boys) with histologic confirmation of PVOD. RESULTS: PH was diagnosed at a mean age of 13.5 years (range, 8-16 years), followed by the definitive diagnosis of PVOD at a mean age of 14.3 years (range, 10-16 years). Symptoms such as decreased exercise tolerance (n = 6) and/or shortness of breath (n = 9) preceded the diagnosis by 21 months on average; the mean survival time after diagnosis was 14 months (range, 0-47 months). CT scans of the lungs showed typical radiologic features. Treatment included supplemental home oxygen (n = 5), diuretics (n = 9), warfarin (n = 4), and pulmonary vasodilators (n = 4). Four children were listed for lung transplantation, and three have undergone transplantation. Eight patients died, including two after lung transplantation. One patient with lung transplant survived with good quality of life. CONCLUSIONS: PVOD is an important differential diagnosis for pediatric patients with PH. CT scanning is a valuable tool to image lung abnormalities; the definitive diagnosis can only be made by examination of lung biopsy specimens, which subjects the patient to additional risk. Early listing for lung transplantation is essential, as the mean survival time is only 14 months.

Pulmonary Veno-Occlusive Disease/epidemiology , Risk Assessment/methods , Adolescent , Age Factors , Biopsy , Child , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Incidence , Lung Transplantation , Male , Ontario/epidemiology , Prevalence , Prognosis , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/surgery , Respiratory Function Tests , Retrospective Studies , Risk Factors , Survival Rate/trends , Tomography, X-Ray Computed
Am Heart J ; 165(5): 770-7, 2013 May.
Article En | MEDLINE | ID: mdl-23622914

BACKGROUND: Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. METHODS: The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. RESULTS: Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. CONCLUSIONS: Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Pulmonary Veins/abnormalities , Pulmonary Veno-Occlusive Disease/etiology , Scimitar Syndrome/surgery , Vascular Surgical Procedures/adverse effects , Adolescent , Adult , Aged , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Incidence , Infant , Infant, Newborn , Male , Massachusetts/epidemiology , Middle Aged , Prognosis , Pulmonary Circulation , Pulmonary Veins/surgery , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/physiopathology , Retrospective Studies , Scimitar Syndrome/diagnosis , Scimitar Syndrome/mortality , Survival Rate/trends , Time Factors , Treatment Outcome , Vascular Surgical Procedures/methods , Young Adult
Eur Respir J ; 40(3): 596-603, 2012 Sep.
Article En | MEDLINE | ID: mdl-22362843

A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations. Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic variables were evaluated. 866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3- and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar. Epidemiology and survival of PAH patients in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although has a similar survival rate.

Hypertension, Pulmonary/mortality , Adult , Aged , Chronic Disease , Female , Humans , Incidence , Male , Middle Aged , Models, Biological , Prevalence , Pulmonary Veno-Occlusive Disease/epidemiology , Registries/statistics & numerical data , Retrospective Studies , Spain/epidemiology , Thromboembolism/epidemiology
Circ Arrhythm Electrophysiol ; 5(1): 52-60, 2012 Feb.
Article En | MEDLINE | ID: mdl-22062795

BACKGROUND: The risk of pulmonary vein narrowing (PVN) after pulmonary vein isolation, using a novel multi-electrode ablation catheter, is unknown. METHODS AND RESULTS: Left atrial volume and PV diameters were compared by computed tomography (CT) before and 3 months after pulmonary vein isolation using duty-cycled phased radio frequency energy (2:1 or 4:1 bipolar/unipolar ratio) in 50 patients. Pulmonary vein diameter was measured in a coronal and axial view at 3 levels (A, ostium; B, 1 cm more distal; C, 2 cm more distal). Moderate PVN was defined as a pulmonary vein diameter reduction of 25 to 50%, and severe PVN as >50%. Left atrial volume decreased by 12±12% (P<0.01). Axial pulmonary vein diameter shortened by a median of 16% (interquartile range [IQR] 28 to 5%), 13% (IQR 25 to 5%), and 9% (IQR 21 to -3%) at level A, B, and C, respectively (P<0.01 for all); coronal pulmonary vein diameter decreased by a median of 16% (IQR 24 to 7%), 11% (IQR 21 to 4%), and 8% (IQR 18 to -2%; P<0.01 for all). Moderate PVN occurred in 30% of the PVs, in 78% of the patients; severe PVN occurred in 4% of the PVs, in 15% of the patients. PV diameter reduction was not related to changes in left atrial volume. CONCLUSIONS: Isolation of the pulmonary veins using a multielectrode ablation catheter and duty cycled phased radiofrequency energy delivery results in a consistent moderate reduction of the PV diameters predominantly at the ostium. Severe PVN in 15% of patients raises concerns about the risk for clinical PV stenosis.

Atrial Fibrillation/surgery , Catheter Ablation/adverse effects , Heart Conduction System/surgery , Pulmonary Veins/surgery , Pulmonary Veno-Occlusive Disease/epidemiology , Atrial Fibrillation/physiopathology , Belgium/epidemiology , Electrocardiography , Female , Fluoroscopy , Follow-Up Studies , Heart Conduction System/physiopathology , Humans , Male , Middle Aged , Phlebography , Postoperative Complications , Prevalence , Prognosis , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/etiology , Risk Factors , Tomography, X-Ray Computed
Expert Rev Respir Med ; 5(2): 217-29; quiz 230-1, 2011 Apr.
Article En | MEDLINE | ID: mdl-21510732

Pulmonary veno-occlusive disease (PVOD) is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered as idiopathic PAH. PVOD and idiopathic PAH share a similar clinical presentation, genetic background and hemodynamic profile. A definite diagnosis of PVOD necessitates a surgical biopsy, but since it represents a high-risk procedure in these patients, it is contraindicated. Therefore, a noninvasive diagnostic approach using chest high-resolution computed tomography, arterial blood gas analysis, pulmonary function tests and bronchoalveolar lavage is helpful to detect PVOD. PVOD is characterized by a poor prognosis and the possibility of developing severe pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice.

Pulmonary Veno-Occlusive Disease/therapy , Humans , Predictive Value of Tests , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Risk Factors , Treatment Outcome
Presse Med ; 40(1 Pt 2): e65-78, 2011 Jan.
Article En | MEDLINE | ID: mdl-21211937

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension that may develop in patients with connective tissue diseases (CTD). Most cases have been reported in patients with systemic sclerosis, though associations with systemic lupus erythematosis and mixed connective tissue disease have also been described. PVOD is characterised by progressive obstruction of small pulmonary veins and venules that leads to increased pulmonary vascular resistance, right heart failure and premature death. Distinguishing PVOD from pulmonary arterial hypertension (PAH) is often difficult, though use of a diagnostic algorithm may improve diagnostic accuracy and preclude recourse to lung biopsy. The finding of normal left-heart filling pressures in the context of radiological studies suggestive of pulmonary oedema is an important diagnostic clue, particularly if this clinical scenario coincides with the introduction of vasodilator therapy. There are no approved treatments for the disorder, though cautious use of PAH specific therapy may improve short-term outcomes in selected idiopathic PVOD cases. This review summarises the epidemiologic, clinico-pathologic and imaging characteristics of PVOD in the setting of CTD and discusses potential management approaches.

Connective Tissue Diseases/complications , Hypertension, Pulmonary/etiology , Pulmonary Veno-Occlusive Disease/etiology , Humans , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/therapy , Risk Factors
Circulation ; 122(25): 2718-26, 2010 Dec 21.
Article En | MEDLINE | ID: mdl-21135364

BACKGROUND: late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. METHODS AND RESULTS: we conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. CONCLUSIONS: preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.

Cardiovascular Surgical Procedures , Postoperative Complications/epidemiology , Pulmonary Veno-Occlusive Disease/epidemiology , Female , Humans , Infant, Newborn , Male , Pulmonary Veins/abnormalities , Retrospective Studies , Risk Factors , Scimitar Syndrome/surgery , Treatment Outcome
Echocardiography ; 27(9): 1141-6, 2010 Oct.
Article En | MEDLINE | ID: mdl-20678129

AIMS: Pulmonary vein (PV) stenosis is a potentially serious complication of catheter ablation of atrial fibrillation (AF). The optimal method for diagnosis of PV stenosis has not been established. We undertook a systematic review of the literature to investigate the diagnostic performance of transesophageal echocardiography (TEE) for the detection of PV stenosis after catheter ablation of AF. METHODS: We searched MEDLINE and EMBASE databases for studies evaluating the diagnostic performance of TEE for the detection of PV stenosis after catheter ablation of AF, compared to a reference standard of PV angiography, magnetic resonance imaging (MRI), or computed tomography (CT). Study quality was assessed using the QUADAS tool. RESULTS: A total of seven studies that included 344 patients (1,344 PVs) met the selection criteria. Of these, three studies used PV angiography as the reference standard, while MRI and CT were used in two studies each. Compared to PV angiography, TEE had sensitivity between 82% and 100% and specificity between 98% and 100%. Compared to MRI, TEE sensitivity was 100% in both studies, while the specificity ranged between 98% and 99%. Compared to CT, TEE had sensitivity between 86% and 100% and specificity of 95%. Quality of the reviewed studies was somewhat limited by the retrospective design in most of the studies. CONCLUSIONS: TEE has a high sensitivity and specificity in detecting PV stenosis. Given its wide availability and favorable side effect profile, these data suggest that TEE is very useful tool for the diagnosis of PV stenosis after catheter ablation of AF.

Atrial Fibrillation/epidemiology , Atrial Fibrillation/surgery , Catheter Ablation/statistics & numerical data , Echocardiography, Transesophageal/statistics & numerical data , Postoperative Complications/epidemiology , Pulmonary Veno-Occlusive Disease/diagnostic imaging , Pulmonary Veno-Occlusive Disease/epidemiology , Atrial Fibrillation/diagnostic imaging , Comorbidity , Female , Humans , Incidence , Male , Risk Assessment , Treatment Outcome
Pediatrics ; 122(3): e656-61, 2008 Sep.
Article En | MEDLINE | ID: mdl-18762500

OBJECTIVE: Pulmonary vein stenosis is a rare, although often lethal, anomaly. Risk factors for the diagnosis of pulmonary vein stenosis are poorly characterized. In this study we sought to identify factors associated with pulmonary vein stenosis, paying particular attention to preterm birth. METHODS: By review of the cardiac database we identified all of the subjects with pulmonary vein stenosis over a 10-year period at our institution. Those children with anomalous pulmonary venous connection were not included. Patient-related variables were analyzed for their association with pulmonary vein stenosis. Pulmonary vein stenosis was diagnosed by spectral Doppler interrogation of the pulmonary veins (continuous, turbulent flow with calculated mean gradient > 5 mm Hg) and confirmed by cardiac catheterization in nearly all of the cases. RESULTS: Twenty-six patients with pulmonary vein stenosis were identified. The median age at diagnosis was 7.4 months; range: 1 day to 35 months. Congenital heart defects were present in the majority of subjects. Associated genetic syndromes were present in 8 subjects (31%). The 2-year survival rate from diagnosis was 43%. The majority of subjects (16 [61%]) were preterm. Gestational ages ranged from 24.2 to 41.0 weeks, and birth weights ranged from 460 to 4445 g. Preterm birth was strongly associated with the diagnosis of pulmonary vein stenosis, odds ratio 10.2 (95% CI 4.7-22.6), p < .001. Eleven (42%) of the 26 subjects were treated for bronchopulmonary dysplasia before being diagnosed with pulmonary vein stenosis. CONCLUSIONS: Prematurity is associated with the diagnosis of pulmonary vein stenosis. It is interesting to note that many of these patients also have intracardiac shunt lesions, which may act in concert with preterm endothelium to produce pulmonary vein stenosis.

Heart Defects, Congenital/diagnosis , Infant, Premature , Pulmonary Veins/abnormalities , Pulmonary Veno-Occlusive Disease/diagnosis , Blood Flow Velocity , Child, Preschool , Diagnosis, Differential , Follow-Up Studies , Georgia/epidemiology , Gestational Age , Heart Defects, Congenital/epidemiology , Humans , Infant , Infant, Newborn , Prevalence , Pulmonary Veins/diagnostic imaging , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/physiopathology , Retrospective Studies , Survival Rate/trends , Ultrasonography, Doppler, Color
Rev. méd. hondur ; 75(1): 30-40, ene.-mar. 2007. ilus
Article Es | LILACS | ID: lil-476359

La tuberculosis es un problema de tal magnitud que la Organización Mundial de la Salud la ha declarado una emergencia global. El porcentaje exacto de niños con tuberculosis se estima que es de 3 a 13% de todos los casos.La patogénesis y el cuadro clínico de la tuberculosis infantil presentan características particulares debido a la inmadurez del sistema inmune y a la escasez de manifestaciones clínicas en el niño aún con tuberculosis activa.El diagnóstico es difícil y se basa en una combinación de criterios: 1-. Contacto con un adulto con tuberculosis.2-. Cuadro clínico sugestivo (anorexia, falla en el medro,fiebre persistente, apatía, etc). 3-. Prueba de tuberculina positiva. 4-. Anormalidades en la radiografía de tórax(adenopatías hiliares, patrón miliar, cavernas, etc). 5-. Baciloscopía o cultivo positiva. 6-. Granuloma específico en resultado de anatomía patológica. El tratamiento se basa en una combinación de Isoniacida, Rifampicina, Pirazinamida y Etambutol (o Estreptomicina) por dos meses,seguido de Isoniacida y Rifampicina por cuatro meses. La cuarta droga puede ser omitida si la drogorresistencia es poco probable o la enfermedad es mínima. La tuberculosis multidrogorresistente puede requerir de cuatro a siete drogas con una duración más prolongada y debe ser manejada por un experto en tuberculosis. La adherencia al tratamiento es esencial para el éxito de la terapia. La prevención se basa en el tratamiento de la enfermedad activa, tratamiento de la infección tuberculosa latente y la vacunación con BCG...

Humans , Male , Female , Child, Preschool , Child , Tuberculosis, Pulmonary/epidemiology , Tuberculosis, Pulmonary/etiology , Antibiotics, Antitubercular/therapeutic use , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , BCG Vaccine/administration & dosage , BCG Vaccine/therapeutic use
J Interv Card Electrophysiol ; 14(1): 21-5, 2005 Oct.
Article En | MEDLINE | ID: mdl-16311935

INTRODUCTION: Pulmonary vein (PV) stenosis is an important complication of the AF ablation and could be underestimated if their assessment is not systematically done. Selective Segmental Ostial Ablation (SSOA) and Circunferential Pulmonary Veins Ablation (CPVA) have demonstrated efficacy in atrial fibrillation (AF) treatment. In this study the real incidence of PV stenosis in patients (pts) submitted to both SSOA and CPVA was compared. METHODS: Those pts with focal activity and normal left atrial size were submitted to SSOA, remaining pts were submitted to CPVA to treat refractory, symptomatic AF. Contrast enhanced magnetic resonance angiography (MRA) was routinely performed in all patients 4 months after the procedure. RESULTS: A series of 73 consecutive patients (mean age of 51 +/- 11 years; 75% male) were included. SSOA was performed in 32 patients, and the remaining 41 patients underwent to CPVA, obtaining similar efficacy rates (72% vs 76% arrythmia free probability at 12 months; log rank test p = NS). Six patients had a significant PV stenosis, all in SSOA group none in CPVA group (18.8% vs 0%; p = 0.005). All patients were asymptomatic and the stenosis was detected in routine MRA. No predictors of stenosis has been identified analysing patient procedure characteristics. CONCLUSION: PV stenosis is a potential complication of SSOA not seen in CPVA. The study confirms than MRA is useful for identifying patients with asymptomatic PV stenosis.

Atrial Fibrillation/surgery , Catheter Ablation/methods , Postoperative Complications/epidemiology , Pulmonary Veins/surgery , Pulmonary Veno-Occlusive Disease/epidemiology , Chi-Square Distribution , Female , Humans , Incidence , Logistic Models , Magnetic Resonance Angiography , Male , Middle Aged , Treatment Outcome
J Am Soc Echocardiogr ; 18(6): 654-9, 2005 Jun.
Article En | MEDLINE | ID: mdl-15947769

OBJECTIVE: Doppler-derived flow velocity measured by transesophageal echocardiography (TEE) may overestimate pulmonary vein stenosis. We hypothesized that combining peak velocity with a stenotic flow pattern improves diagnosis compared with magnetic resonance imaging (MRI). METHODS: TEE and MRI were performed in 44 patients 19 +/- 11 months after radiofrequency catheter ablation. Pulmonary vein stenosis was defined by a peak velocity of 110 cm/s or more plus a stenotic flow pattern (turbulence and reduced flow variation) on TEE and a lumen reduction of more than 50% on MRI. RESULTS: In all, 175 pulmonary veins were studied. MRI showed 7 cases of pulmonary vein stenosis that were correctly identified by TEE. In addition, TEE criteria for pulmonary vein stenosis were met in 4 pulmonary veins that did not show obstruction on MRI. In all, 5 pulmonary veins with normal appearance on MRI had peak velocity of 110 cm/s or more with normal flow pattern. CONCLUSIONS: TEE Doppler measurements can be reliably used to detect or exclude significant pulmonary vein stenosis if the diagnosis is restricted to a combination of elevated peak velocity (> or = 110 cm/s) with turbulence and little flow variation.

Catheter Ablation/statistics & numerical data , Echocardiography, Transesophageal/statistics & numerical data , Image Enhancement/methods , Magnetic Resonance Imaging/statistics & numerical data , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Female , Germany/epidemiology , Humans , Male , Middle Aged , Prognosis , Reproducibility of Results , Risk Assessment/methods , Risk Factors , Sensitivity and Specificity , Treatment Outcome
Acad Radiol ; 11(11): 1211-8, 2004 Nov.
Article En | MEDLINE | ID: mdl-15561567

RATIONALE AND OBJECTIVES: Pulmonary vein (PV) stenosis is a common complication after radiofrequency ablation for atrial fibrillation. This study investigated the intra- and interobserver variability and precision of PV ostial measurements from three-dimensional computed tomography angiography. MATERIALS AND METHODS: Four observers measured the maximum and minimum diameters, as well as area, of the four PVs of seven patients who underwent a three-dimensional computed tomography scan before radiofrequency ablation. Each observer performed two sets of measurements. The intra- and interobserver variability of the measurements was calculated using analysis of variance. RESULTS: Intraobserver variability was approximately two times lower than interobserver variability in measurements of diameter and area. The standard error of the measurement (SEM), SEM(intra) and SEM(inter), were lower for the mean diameter than the maximum diameter. The minimum detectable changes in diameters, DeltaD(intra) and DeltaD(inter), and area, DeltaA(intra) and DeltaA(inter), demonstrated the same statistical trend as the corresponding SEM for each of the four veins. Both DeltaD(intra) and DeltaD(inter) and DeltaA(intra) and DeltaA(inter) were smaller than the corresponding lower bounds of the 95% confidence interval for 50% diameter reduction and 75% area reduction. The direct results of DeltaA(intra) and DeltaA(inter) for each of the four veins were consistently less than DeltaA's calculated from the corresponding DeltaD(intra) and DeltaD(inter). CONCLUSION: PV ostial measurements are less variable when made by a single observer than by multiple observers, and mean diameter measurements are more precise than a single, maximum diameter measurement. Both diameter and area measurements are capable of quantifying the mild PV stenosis. Furthermore, area can be measured with greater precision than mean diameter and should be used in PV ostium caliber determination.

Angiography , Pulmonary Veins/diagnostic imaging , Tomography, X-Ray Computed , Adult , Aged , Analysis of Variance , Female , Humans , Imaging, Three-Dimensional , Male , Middle Aged , Observer Variation , Pulmonary Veins/pathology , Pulmonary Veno-Occlusive Disease/diagnostic imaging , Pulmonary Veno-Occlusive Disease/epidemiology , Research Design
J Am Coll Cardiol ; 43(12 Suppl S): 5S-12S, 2004 Jun 16.
Article En | MEDLINE | ID: mdl-15194173

In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary pulmonary hypertension" and to replace it with "idiopathic pulmonary hypertension"; to reclassify pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for pulmonary arterial hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-pulmonary shunts.

Hypertension, Pulmonary/classification , Genetic Predisposition to Disease , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/epidemiology , Pulmonary Veno-Occlusive Disease/classification , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Risk Factors