Chest
Volume 117, Issue 1, January 2000, Pages 31-38
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Clinical Investigations
PULMONARY VASCULATURE
Diffuse Pulmonary Arteriovenous Malformations: Characteristics and Prognosis

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Objective

To study the clinical characteristics andprognosis of patients with diffuse pulmonary arteriovenousmalformations (AVMs).

Design

Retrospective chartreview of all patients (n = 16) with diffuse pulmonary AVMs seen at Yale New Haven Hospital, Johns Hopkins Hospital, and St. Michael's Hospital. Up-to-date follow-up information was obtained in all livingpatients.

Results

All patients wereseverely hypoxic. Neurologic complications (stroke or brain abscess)had occurred in 70% of patients by the time of diagnosis. During thefollow-up period (mean, 6 years), three patients died and two othersdeveloped new neurologic complications. One of the deaths occurredperioperatively during lung transplantation. All patients underwenttranscatheter embolotherapy of any large pulmonary AVMs. A selectedgroup underwent pulmonary flow redistribution, a novel technique.Oxygenation did not improve significantly with embolotherapy of thelarger AVMs, but there was a small significant improvement in thosepatients who underwent pulmonary flow redistribution. The majority(85%) of the living patients are currently working or studyingfull-time.

Conclusions

Patients with diffusepulmonary AVMs are at increased risk of neurologic complications.Transcatheter embolotherapy does not significantly improve the profoundhypoxia, but it may reduce the risk of neurologic complications.Antibiotic prophylaxis is recommended for bacteremic procedures toprevent brain abscess. These patients can live for many years and leadproductive lives. We do not recommend lung transplantation becausesurvival with disease is difficult to predict and we have observed aperioperative transplant death.

Section snippets

Materials and Methods

All patients with pulmonary AVMs (n = 351) who were seen in three hospitals (Saint Michael's Hospital, Yale New Haven Hospital, and Johns Hopkins Hospital) between 1980 and 1998 were screened for this study. The patients who were selected included only those with diffuse pulmonary AVMs, defined as AVMs involving every subsegmental artery of at least one lobe (Fig 1). This is contrasted with the more frequent presentation of multiple discrete pulmonary AVMs (Fig 2). Five of the patients

Results

Of the 351 pulmonary AVM patients who were identified during the 18-year interval, 16 patients (5%; 10 female and 6 male) had diffuse pulmonary AVMs (Table 1). Of these, HHT was definitively diagnosed in 12 patients (75%) and was suspected in 2 patients (13%). Although many had epistaxis in this group, only one had GI bleeding. Cerebral AVMs were not diagnosed in any of the patients. Interestingly, some had a family history of discrete pulmonary AVMs but none had a family history of diffuse

Discussion

Pulmonary AVMs are known to cause serious complications. These complications can be prevented if AVMs are managed appropriately with TCE. Little is known about the subgroup of patients with diffuse pulmonary AVMs. Many of the reported patients had a poor prognosis, although few of them had undergone any specific therapy. Their severe hypoxia and our inability to improve it with TCE has led some researchers to suggest that these patients undergo lung transplantation. We have demonstrated that

Conclusion

Patients with diffuse pulmonary AVMs have a fairly good prognosis and can lead productive lives. In a select group of these patients, small improvements in oxygenation can be obtained with PFR. However, patients with diffuse pulmonary AVMs are at increased risk for neurologic complications; therefore, antibiotic prophylaxis and TCE of the larger AVMs is of utmost importance.

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    This work was supported by a fellowship (for Dr. Faughnan) from the Canadian Lung Association/Glaxo/Medical Research Council of Canada, aswell as funding from the Nelson Arthur Hyland Foundation. Dr. Robert I.White, Jr. is supported in part by a grant from the Josephine Laurence Hopkins Foundation.

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