Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Drainage: Role of Primary Sutureless Technique.

To evaluate the surgical outcomes of total anomalous pulmonary venous drainage focusing on survival, postoperative and pulmonary venous obstruction. Further investigate the role of primary sutureless technique in patients with preoperative pulmonary venous obstruction. Consecutive patients underwent total anomalous pulmonary venous drainage repair in our institution during Jan 2000 to Dec 2019 were enrolled into this retrospective analysis. Since 2016, sutureless repair was regularly applied in patients with preoperative pulmonary venous obstruction. All patients with preoperative pulmonary venous obstruction referred before 2016 had underwent traditional repair. A total of 95 patients were included. During follow-up time of 85 months, main endpoints were documented in 21 patients, including 9 (9.5%) early deaths, 3 (2.3%) late deaths and 9 (9.5%) postoperative pulmonary venous obstructions. Preoperative pulmonary venous obstruction was presented in 26 (27.4%) patients with more emergent surgery (14/26 vs 3/69, P < 0.001) was required. Main endpoints occurred more in patients with preoperative pulmonary venous obstruction (4/26 vs 5/69, P = 0.004). Patients experienced sutureless technique had a lower incidence of postoperative PVO at follow-up (0/11 versus 4/11, P = 0.045). Outcomes of surgical repair for total anomalous venous drainage are satisfactory. However, preoperative pulmonary venous obstruction may be accompanying unfavorable early deaths and postoperative pulmonary venous obstruction. Propensity matching analysis showed that sutureless technique was benefit for postoperative pulmonary venous obstruction without longer cardiopulmonary bypass and aortic cross-clamp time.

Single-institution outcomes of surgical repair of infracardiac total anomalous pulmonary venous connection.

OBJECTIVE: This contemporary study sought to describe the outcomes of patients undergoing biventricular repair of infracardiac total anomalous pulmonary venous connection. METHODS: A retrospective study was performed on patients with infracardiac total anomalous pulmonary venous connection who underwent sutureless technique or conventional repair between 2006 and 2018. Risk factors for survival and post-repair pulmonary vein stenosis (PVS) were assessed with Cox regression model. Time-to-event analysis was conducted using Kaplan-Meier estimates. RESULTS: This study included 82 consecutive patients with the median age of 21 days (interquartile range, 9-40 days). The median follow-up was 29 months (interquartile range, 12.5-59 months) and was available in 95% of the survivors at the end of the study period in 2019. Overall, 8 deaths (8.5%) occurred in the conventional repair group. There was a trend of higher mortality in the conventional repair group, although it did not reach a statistical difference (P = .2). Postrepair PVS occurred at a median of 2 months (interquartile range, 1.2-3.6 months) postoperatively and all occurred in the conventional repair group. Time-to-event analysis with the event of postrepair PVS showed significantly higher freedom from restenosis in the sutureless technique group (P = .0004). Adjusted hazard ratios from time-dependent Cox model described the association between postrepair PVS and pulmonary venous confluence of antler configuration (hazard ratio, 2.14; 95% confidence interval, 1.03-5.47; P = .002) and the use of sutureless technique (hazard ratio, 0.72; 95% confidence interval, 0.39-0.97; P = .003). CONCLUSIONS: Sutureless technique is associated with a lower risk of postrepair PVS in patients with infracardiac total anomalous pulmonary venous connection. pulmonary venous confluence configuration of antler appearance appears to be associated with restenosis and mortality.

Pharmacovigilance in a rare disease: example of the VIGIAPATH program in pulmonary arterial hypertension.

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.

[Pulmonary veno-occlusive disease]. / La maladie veino-occlusive pulmonaire.

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.

Long-term outcomes of total correction for isolated total anomalous pulmonary venous connection: lessons from 50-years' experience.

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.

Respiratory effects of trichloroethylene.

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 exposure to organic solvents: a risk factor for pulmonary veno-occlusive disease.

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.

Pulmonary venoocclusive disease in childhood.

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.

Outcome predictors and implications for management of scimitar syndrome.

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 veno-occlusive disease: advances in clinical management and treatments.

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: the bête noire of pulmonary hypertension in connective tissue diseases?

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.

Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study.

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.

Pulmonary vein stenosis: prematurity and associated conditions.

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.

Tuberculosis infantil: un enfoque actual / Childhood tuberculosis a current approach

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...

Clinical classification of pulmonary hypertension.

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.

Intraoperative and percutaneous stenting of congenital pulmonary artery and vein stenosis.

BACKGROUND: Conventional surgical or balloon dilation therapy for pulmonary artery or vein stenosis has been unsatisfactory in many patients. Balloon-expandable stents offer a new form of treatment for these vascular stenoses and can be implanted percutaneously or intraoperatively. METHODS AND RESULTS: Between July 1991 and October 1992, 20 balloon-expandable Palmaz stents (Johnson & Johnson) were implanted in 16 children at median age and weight of 3.0 years and 12.8 kg, respectively. Stent implantation was performed intraoperatively (n = 15) if the patient was less than 1 year of age or less than 10 kg in weight, in cases where limited vascular access precluded percutaneous implantation, or as an adjunct to other intracardiac surgery. Otherwise, percutaneous stenting was performed (n = 5). Vessels were tested for distensibility by dilation with balloon catheters or vascular sounds. Stents were implanted using angioplasty catheter balloons chosen to achieve desired vessel diameter and inflated to 4 to 17 atm. Acute hemodynamic and cineangiographic studies were performed in all patients immediately after the procedure to 2 months after stenting. After pulmonary artery stent implantation, mean pulmonary artery diameter increased from 5.6 to 11.5 mm (P = .001), with a decrease in mean systolic pressure gradients from 43 to 8.0 mm Hg (P = .005). Follow-up cardiac catheterization (mean, 8.7 months) in 3 patients revealed no restenosis, thrombosis, or aneurysm formation. In patients in whom pulmonary vein stents were implanted, mean pressure gradients fell from 11 to 0.3 mm Hg (P = .03), and mean pulmonary capillary wedge pressure fell from 17 to 6.3 mm Hg (P = .03) immediately after stenting. At 2- to 6-month follow-up, cardiac catheterization documented restenosis within the stent in 2 of 3 patients. The third patient died 2 months after stenting from presumed vein reocclusion. CONCLUSIONS: When implanted intraoperatively or percutaneously, balloon-expandable endovascular stents have been efficacious in the treatment of pulmonary artery stenosis. Longer follow-up will be necessary to document the long-term effectiveness of pulmonary artery stenting. Preliminary data suggest that early restenosis is common after pulmonary vein stenting. The intraoperative approach extends stenting therapy to smaller children and to patients who have limited percutaneous access.

Pulmonary wedge angiography for prediction of pulmonary vascular disease in Down syndrome.

We performed high resolution pulmonary wedge angiography (PWA) and conventional hemodynamics to predict the reversibility of structural pulmonary vascular disease. Sixty-one pulmonary wedge angiograms were performed on 41 patients with intracardiac shunts and Down syndrome (median age 8 months). Balloon occlusion wedge angiograms were analyzed for (1) monopedial branches from the distal 10 mm of muscular arteries, (2) capillary blush, (3) tapering indices, and (4) tortuosity. Twenty-five patients had open lung biopsy, graded by the Health Edwards classification, and analyzed morphometrically. Pulmonary vascular resistance of > or = 6 units was 100% sensitive and 94% specific for Heath Edwards Grade III-IV. A monopedial count < 3 vessels was 83% sensitive and 100% specific for Heath Edwards Grade III-IV. Abnormal capillary blush was 83% sensitive and 69% specific for Heath Edwards Grade III-IV. Tapering indices and tortuosity showed no significant correlation with lung biopsy. A combination of pulmonary vascular resistance < 6 units, monopedial count > or = 3, and normal capillary blush was 100% sensitive and specific for Heath Edwards Grade 0-II, and a combination of pulmonary vascular resistance > or = 6 units, monopedial count < 3, and abnormal capillary blush was 100% sensitive and specific for Heath Edwards Grade III-IV. Using the 3 criteria, Heath Edwards Grade was accurately predicted in 17 patients. In 4 patients, only 2 criteria were available. Morphometric analysis showed an inverse relationship between the lowest monopedial count and the number of occlusive vessels per cm of tissue, r = -0.74 p < 0.001. Arteries showing intimal and/or medial thickening causing > 90% luminal narrowing were scored as "occlusive." These results show that when the hemodynamic and pulmonary wedge angiography data are concordant, the structural changes of pulmonary vascular disease can be accurately predicted and lung biopsy may be avoided.