The index of pulmonary vascular disease in children with congenital heart disease: relationship to clinical and haemodynamic findings

Gorenflo, Matthias; Ullmann, Michael V.; Sebening, Christian; Brockmeier, Konrad; Hagl, S.; He, Ulmer; Otto, H. F.; Schnabel, P.

doi:10.1007/s00428-002-0647-4

Cited by 9 publications

(7 citation statements)

References 25 publications

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“…15 The index of pulmonary vascular disease significantly decreased between PAB and ICR (p=0.0469). It improved at ICR in patients with PVOD, and also tended to improve at ICR in the AVSD patients with PVOD.…”

Section: Discussionmentioning

confidence: 91%

“…Pulmonary vascular disease was evaluated according to the index of pulmonary vascular disease of Yamaki et al 14,15 and the subsequent definition of advanced PVOD. The following parameters were compared between patients with and without PVOD: pulmonary-to-systemic flow ratio (Qp/Qs) and pulmonary-to-systemic pressure ratio (Pp/Ps) from the data obtained during cardiac catheterization performed under continuous sedation before PAB and before ICR, and the cardiothoracic ratio (CTR) on chest X-ray taken before PAB (Table 2).…”

Section: Methodsmentioning

confidence: 99%

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Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Ohashi

Matsushima

Maeda

et al. 2006

Circ J

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Background Down syndrome patients are characterized by early progression of pulmonary vascular obstructive disease because of insufficient thickness of the pulmonary arterial media. For those with congenital heart disease (CHD) associated with pulmonary hypertension (PH), a 2-stage procedure of pulmonary artery banding (PAB) and then intracardiac repair (ICR) in early infancy is performed to prevent such pulmonary vascular diseases in early infancy. Methods and ResultsThe subjects were 16 patients with Down syndrome who underwent lung biopsy during PAB and ICR. PAB was planned to be performed in early infancy and ICR approximately 1 year later. Efficacy of the 2-stage procedure was retrospectively examined with reference to pulmonary vascular disease and pulmonary diseases. The index of pulmonary vascular disease at PAB fulfilled the indication for ICR, and it was significantly lower at ICR than at PAB (p=0.0469); furthermore, PAB prevented progression of pulmonary diseases. Conclusions The results of the lung biopsies support the safety of the 2-stage procedure and show that it is effective for Down syndrome patients with CHD and PH. (Circ J 2006; 70: 1446 -1450

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Section: Discussionmentioning

confidence: 91%

Section: Methodsmentioning

confidence: 99%

Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Ohashi

Matsushima

Maeda

et al. 2006

Circ J

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“…Moreover, the cases described in the present report are of particular clinical interest, since both MPV/ACD and ventricular septal defects are accompanied by pulmonary hypertension. In the case of pulmonary hypertension due to a ventricular septal defect, histological examination shows media hypertrophy of muscular arteries, muscularization of arterioles, and plexiform lesions, but not misalignment of lung vessels or alveolar dysplasia [5]. Moreover, although morphological changes in bronchopulmonary dysplasia in the infant respiratory distress syndrome (IRDS) can resemble those in ACD, the reduction (or even loss) of capillaries is much more prominent in ACD.…”

Section: Discussionmentioning

confidence: 98%

Misalignment of pulmonary vessels with alveolar capillary dysplasia: association with atrioventricular septal defect and quadricuspid pulmonary valve

et al. 2005

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Misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia (ACD) is a complex congenital vascular abnormality in newborn infants. This condition is often associated with persistent pulmonary hypertension, which leads to severe respiratory distress and finally to the death of the newborn. Here, two cases of MPV with ACD are reported that were associated with atrioventricular septal defects. Both cases showed a combination of a large atrial septal defect and a smaller subvalvular ventricular septal defect. In one newborn, the cardiac malformation was accompanied by a quadricuspid pulmonary valve. These cases emphasize the importance of considering MPV with ACD a possible cause for pulmonary hypertension in the newborn, especially when a concomitant atrioventricular septal defect could represent an alternative explanation for the pulmonary hypertension.

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“…Nineteen domestic farm piglets (German Landrace) 4 weeks of age and with a body weight of 12 kg [10][11][12][13][14][15][16] [median (range)] were used. The piglets were intubated and ventilated with a respirator (Servo Ventilator 900C, SiemensElema, Umea, Sweden) using tidal volumes of 10 to 15 ml/kg and a respiratory rate of 12 to 15 breaths per minute.…”

Section: Surgical Proceduresmentioning

confidence: 99%

“…The pathological changes in humans are characterized by medial hypertrophy and intimal proliferation finally leading to plexiform lesions and obliterative intimal fibrosis [14]. Both impaired endogenous nitric oxide synthesis [4] and an increase in pulmonary endothelin-1 (ET-1) [11] have been associated with pulmonary vascular changes in humans.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary vascular changes in piglets with increased pulmonary blood flow and pressure

et al. 2007

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In this model of pulmonary vascular disease, high pulmonary blood flow was created by an anastomosis between the left subclavian artery and the main pulmonary artery [Blalock-Taussig (BT) shunt] in 4-week-old piglets (n = 6). Additional ligation of the left pulmonary artery (LPA) was used to increase pulmonary artery pressure (n = 6). Seven piglets were sham-operated. After 3 months, mean pulmonary artery pressure was higher in animals with BT shunt and LPA ligation (22 +/- 5; mean+/-SD) compared to sham-operated animals (15 +/- 2). In addition, thickening of the medial coat (20.1 +/- 2.8% versus 13.6 +/- 3.1% wall thickness) and increased immunostaining for vascular endothelial growth factor A (VEGF-A) were observed. Relative gene expression for endothelin-converting enzyme-1 (ECE-1) mRNA was 1.8 times higher, and VEGF-A mRNA was 2.5 times higher in pigs with BT shunt and LPA ligation compared with sham-operated animals. VEGF receptor-1 and VEGF receptor-2 mRNA was lower in shunted animals and in animals with additional ligation of LPA. Upregulation of ECE-1 and VEGF-A, as well as changes in VEGFR expression in the pulmonary hypertensive lung, may contribute to pulmonary vascular changes.

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The index of pulmonary vascular disease in children with congenital heart disease: relationship to clinical and haemodynamic findings

Abstract: The IPVD is not helpful to predict surgical mortality during the first year of life. Patients with trisomy 21 and cAVSD may show advanced pulmonary vascular disease in infancy.

Cited by 9 publications

References 25 publications

Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Misalignment of pulmonary vessels with alveolar capillary dysplasia: association with atrioventricular septal defect and quadricuspid pulmonary valve

Pulmonary vascular changes in piglets with increased pulmonary blood flow and pressure

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