Diagnosis of pulmonary venous obstruction by Doppler echocardiography

A 1 1/2-year-old female patient with atrial and ventricular septal defects and severe pulmonary arterial hypertension was found to have discrete stenosis of left upper and middle lobe pulmonary veins. The diagnosis was made by cross-sectional and Doppler echocardiography. The findings were confirmed at cardiac catheterization and operation. Pressure gradients across the pulmonary vein stenosis as obtained by Doppler echocardiography correlated well with those measured at cardiac catheterization.     

Unilateral pulmonary edema due to pulmonary venous obstruction from fibrosing mediastinitis

An unusual case of fibrosing mediastinitis with obstruction of the inferior and superior left pulmonary veins and severe narrowing of the right pulmonary artery, disclosed after unilateral pulmonary edema, is described. The 18-year-old male patient had a long history of cough, progressive dyspnea and recurrent hemoptysis and the possible diagnosis of "interstitial fibrosis" from a previous lung biopsy. The diagnosis and the pulmonary vessels involvement were suspected after right heart catheterization combined with transesophageal echocardiography and confirmed during urgent thoracotomy and at postmortem examination.     

Magnetic resonance phase-shift velocity mapping in pediatric patients with pulmonary venous obstruction.

OBJECTIVES: This study evaluated the accuracy, advantages and clinical efficacy of magnetic resonance (MR) phase-shift velocity mapping, in delineating the site and the hemodynamic severity of pulmonary venous (PV) obstruction in patients with congenital heart disease (CHD). BACKGROUND: Magnetic resonance phase-shift velocity mapping of normal pulmonary veins and of obstructed PV pathways have been previously reported in a mainly adult population. METHODS: The study population (33 pts) underwent MR phase-shift velocity mapping of their PV pathways. These results were compared with cardiac catheterization and Doppler echocardiography data. RESULTS: The study population (0.4 to 19.5 years) consisted of a study group (PV pathway obstruction, n = 7) and a control group (no PV obstruction, n = 26). No patients had any left-to-right shunt lesions. The MR imaging displayed precise anatomical detail of the pulmonary veins. Phase velocities in the control group ranged from 20 to 71 cm/s, whereas velocities in the study group ranged from 100 to 250 cm/s (p = 0.002). The MR phase velocities (154 +/- 0.53 cm/s) compared favorably with Doppler echocardiography (147 +/- 0.54 cm/s), (r = 0.76; p = 0.05). The MR velocity mapping was 100% specific and 100% sensitive in detecting PV obstruction, although the absolute gradient measurements among MR phase mapping, echocardiographic Doppler and catheterization did not show statistically significant correlation. CONCLUSIONS: In the absence of any associated left-to-right shunt lesions, PV velocities of 100 cm/s and greater indicated significant obstruction. The MR phase-shift velocity mapping, together with MR spin echocardiography and MR angiography, provides comprehensive anatomic and physiologic data that may obviate the need for further invasive studies.     

Successful release of recurrent pulmonary venous obstruction after repair of totally anomalous pulmonary venous connection by transcatheter implantation of stents

We report a 3-month-old female infant, in whom pulmonary venous obstruction occurred after repair of totally anomalous pulmonary venous connection, and which was successfully released by a transcatheter implantation of a stent using the transseptal approach. Close follow-up is required, since the long-term outcome is still unclear. Nevertheless, transcatheter implantation of stents is a promising option for treating this challenging lesion.     

The pulmonary venous wedge pressure in pulmonary arterial hypertension

The comparability of the main pulmonary artery pressure (PAP) and the pulmonary venous wedge pressure (PVWP) was assessed during cardiac catheterization in 89 patients with pulmonary artery hypertension (PAH) and increased pulmonary blood flow. Preliminary evaluation revealed a wide disparity between the 2 determinations. Fifty-five pull-back pressure recordings from branch-to-main pulmonary artery were analyzed. Twenty-four percent (13/55) had systolic pressure gradients >20 mm Hg. between branch and main pulmonary artery. When PVWP and only ipsilateral branch PAP were compared (n=48), diastolic and mean (m), but not systolic PVWP, correlated closely with branch PAP (r=0.77, r=0.73 and r=0.59, respectively). In 46 of 48 patients the PVWP_m was not significantly greater than the ipsilateral PAP. Twenty-nine of 30 patients with PVWP_m <30 mm Hg. had an ipsilateral PAP_m <40 mm Hg. In 15 patients with PVWP_m between 30 and 39 mm Hg, there was a wide range (30–59 mm Hg) of PAP_m. Three patients with PVWP_m >40 mm Hg. had severe PAH. It is concluded that: 1) hemodynamically significant branch-to-main PAP gradients are present in some patients with PAH and may result in erroneously high pulmonary arteriolar vascular resistance when calculated from main PAP; 2) properly performed PVWP determination can define the lower limit of mean pressure in the ipsilateral branch pulmonary artery; 3) a PVWP_m <30 mm Hg. usually indicates an ipsilateral PAP_m <40 mm Hg; 4) a PVWP_m >30 mm Hg. is compatible with either moderate or severe PAH; 5) correlation of PVWP with PAP is not related to pulmonary blood flow.     

Balloon dilation of pulmonary venous pathway obstruction in an infant after the mustard procedure

Pulmonary venous pathway obstruction developed after a Mustard procedure in an infant with transposition of the great arteries. The stenosis was successfully dilated by the percutaneous balloon technique with immediate clinical, echocardiographic, and hemo-dynamic improvement, which has persisted for 8 months after dilation. This technique is a feasible alternative to reoperation in such an obstruction, even in infants.     

Pulmonary hypertension secondary to partial pulmonary venous obstruction in a child with Cantu syndrome

We report on an African‐American male with Cantu syndrome who required a pericardial window for a significant pericardial effusion in infancy and was subsequently found to have partial pulmonary venous obstruction (PVO) leading to pulmonary hypertension. Measurement of bilateral pulmonary capillary wedge pressures is important to uncover partial PVO. Pediatr Pulmonol. 2010; 45:727–729. © 2010 Wiley‐Liss, Inc.     

Regional esophageal pressures with lobar obstruction in dogs

The right lower lobes (RLL) of intact horizontal dogs were obstructed at FRC during spontaneous breathing and artificial ventilation (IPPB). At end inspiration, pressure within the RLL became less than tracheal pressure, and pressure in the lower esophagus declined to a greater extent than in the upper esophagus. Tidal pressure swings in the obstructed RLL were larger than in the esophagus during spontaneous breathing but smaller during IPPB implying that the elastic recoil of the RLL decreased during inspiration at the mediastinal side. All these changes were intensified during RLL atelectasis and counteracted by strapping of the lower chest wall. These results indicate that: (a) around a lobe moving out of phase with the rest of the lung, a force is created tending to minimize the lobar volume differences; (b) the obstructed lobe is deformed during inflation of the rest of the lung; (c) esophageal (and hence pleural) pressure gradient is modified by this localized intrapulmonary pathology apparently due to shape interactions between chest wall and lung.     

Stenting for SVC obstruction in an infant operated for total anomalous pulmonary venous return.

Superior vena cava obstruction following corrective repair of total anomalous pulmonary venous return has rarely been described in the literature. A one-month-old boy who underwent corrective surgery for obstructive supracardiac total anomalous pulmonary venous return with consequent symptomatic superior vena cava obstruction in the immediate postoperative period, is reported. This was treated by balloon dilatation followed by stenting of the superior vena cava. The immediate postoperative result was satisfactory and the infant continued to remain asymptomatic at six months follow up. We suggest that this intervention could prove to be a viable alternative to a repeat surgical procedure for such complex cases.