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Category Archives: Pulmonary Function
Osler-Weber-Rendu Disease and Pulmonary Arteriovenous Fistulas: Discussion (1)
Our case documents hypoxemia and intrapleural rupture of an AVF in a young woman in her 24th week of pregnancy. She had been asymptomatic prior to pregnancy, suggesting a causal relationship. Complicating her illness was the presence of rheumatic mitral valve disease and congestive heart failure.
Hemothorax is a rare but potentially fatal complication of pulmonary AVFs. There have been 16 reported cases of rupture into the pleural space, including our own. Of the 14 in which outcome was reported, sudden death occurred in five. In addition, there are two reports of hemothorax due to the rupture of pleural telangiectasia.' Interestingly, 11 of 13 cases in which the location of the hemothorax was mentioned were left-sided—the reason for this finding is unclear.
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Osler-Weber-Rendu Disease and Pulmonary Arteriovenous Fistulas (3)
She initially stabilized with diuresis, afterload reduction and supplemental oxygen. However, two weeks after admission she developed worsening pulmonary edema and hypoxemia with a Po2 value of 51 mm Hg while breathing 100 percent Os. She was intubated, mechanically ventilated and treated for congestive heart failure. Complete pulmonary angiography followed by coil spring occlusion of her AVFs was performed. Two large and two small right lower lobe AVFs and the previously detected left lower lobe AVF were selectively catheterized and successfully occluded (Fig 2).
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Osler-Weber-Rendu Disease and Pulmonary Arteriovenous Fistulas (2)
Past medical history was remarkable for rheumatic heart disease as a child. Family history was remarkable for recurrent nosebleeds in her father and two children.
Physical examination was notable for diminished breath sounds in the left lung base, fine rales in the right lung base, and a 3/6 mitral regurgitation murmur. Instances of telangiectasia were noted on the lips and buccal mucosa. Digital clubbing was present.
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Osler-Weber-Rendu Disease and Pulmonary Arteriovenous Fistulas (1)
Pulmonary AVFs occur in approximately 15 percent of patients with Osler-Weber-Rendu disease and bleeding complications, such as hemoptysis and rarely hemothorax, are recognized. Several case reports also have associated complications of pulmonary AVFs with pregnancy. We report the case of a young woman with previously undiagnosed Osler-Weber-Rendu disease who presented in the 24th week of pregnancy with hypoxemia and a spontaneous hemothorax due to intrapleural rupture of a pulmonary AVF. She underwent successful coil spring occlusion of her AVFs, the first reported use of embolotherapy in a pregnant patient.
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Serendipitous Discovery During Jugular Catheterization: Discussion (2)
Total anomalous pulmonary venous return typically presents in infancy with congestive heart failure. In contrast, partial anomalous pulmonary venous connection (PAPVC) may be asymptomatic and found incidentally on chest roentgenogram or during evaluation of a murmur, or even be an incidental finding at autopsy; PAPVC is often associated with atrial septal defect and occasionally with complex cardiac defects. Isolated PAPVC in association with intact atrial septum remains unusual and may join the venous circulation at various locations. Drainage of the left upper lobe via a vertical vein to the brachiocephalic vein, as in our patient, is the most common pattern.
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Serendipitous Discovery During Jugular Catheterization: Discussion (1)
The frequency of aberrant placement of central venous catheters using the internal jugular vein as the insertion site varies between 0.8 percent and 5.7 percent. The oxygen saturation of blood from the catheter tip in our patient is consistent with its position in a pulmonary vein draining a lung ventilated with 60 percent oxygen. The pulsatile nature of the waveform is explained on the basis of a catheter whose tip is wedged into a pulmonary venous branch. This converts the pressure tracing from that of the pulmonary vein to that of the upstream pulmonary artery, as has been demonstrated in animal experiments and human studies. The tracing in Figure 1 no longer demonstrates pulmonary hypertension, probably due to the patients preterminal, severe hypoperfusion state. It is interesting that, in retrospect, the high thermodilution cardiac output and elevated mixed venous oxygen saturation seen at pulmonary artery catheterization and attributed to sepsis, may have been partly due to his left-to-right shunt.
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Serendipitous Discovery During Jugular Catheterization (2)
This revealed moderate pulmonary hypertension (68/44 mm Hg with pulmonary capillary wedge pressure of 10 mm Hg), a mixed venous oxygen saturation of 80 percent, and a thermodilution cardiac output of 9 L/min consistent with evolving Pseudomonas sepsis. A complicated and stormy course followed, with myocardial infarction, refractory bacterial and candidal sepsis, massive gastrointestinal hemorrhage, and acute renal failure. On the 39th hospital day a venous introducer sheath and venous catheter were percutaneously placed through the left internal jugular vein without difficulty. A pressure tracing from the tip of the catheter is shown in Figure 1, and this led to concern about the catheters position. The patient was being mechanically ventilated and was on an inspired oxygen fraction of 0.6.
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Serendipitous Discovery During Jugular Catheterization (1)
Placement ot central venous catheters has become an increasingly common accompaniment of modern medicine. The frequent need for central pressure monitoring, hyperalimentation, and long-term venous access leads large numbers of patients to undergo this invasive procedure. It is surprising that the incidence of complications is relatively low and that malposition of catheters is rare. This case describes the first English-language report (to our knowledge) of the serendipitous discovery of an anomalous pulmonary venous connection during placement of a jugular catheter. Of particular interest, this anomaly may have contributed, at least in part, to our patients pulmonary hypertension.
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