eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Arteriovenous Fistulae, Pulmonary: Follow-up

Author: Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital
Contributor Information and Disclosures

Updated: Apr 28, 2009

Follow-up

Deterrence/Prevention

  • See Medication for the recommended prophylactic regimen for dental, oral, sinus, and genitourinary, and GI procedures in patients with pulmonary arteriovenous malformations (PAVMs).
    • Amoxicillin 3 g orally (PO) 1 hour or 2 g intravenously (IV) 30 minutes before the procedure, followed by 1.5 g PO/IV hours after the initial dose
    • For patients who are allergic to penicillin, erythromycin 1000 mg PO 2 hours before the procedure, followed by 500 mg PO 6 hours after the initial dose
    • For patients who are allergic to penicillin, clindamycin 300 mg PO 1 hour or 300 mg IV 30 minutes before procedure, followed by 150 mg PO/IV 6 hours after initial dose
  • The recommended prophylactic regimen for genitourinary and GI procedures includes one of the following:
    • Amoxicillin 3 g PO 1 hour before the procedure, followed by 1.5 g PO 6 hours after the initial dose, plus gentamicin 1.5 mg/kg IV 1 hour before the procedure; this may be repeated 8 hours after the initial dose.
    • For patients who are allergic to penicillin, vancomycin 1 g IV over 1 hour plus gentamicin 1.5 mg/kg IV 1 hour before the procedure; this may be repeated 8 hours after the initial dose.

Complications

  • Seizure
  • Migraine headaches
  • Transient ischemic attack
  • Cerebral vascular accident
  • Brain abscess
  • Hypoxemia, orthodeoxia
  • Hemothorax
  • Life-threatening hemoptysis
  • Pulmonary hypertension
  • Congestive heart failure
  • Polycythemia
  • Anemia
  • Infectious endocarditis

Patient Education

  • Thoroughly educate patients with pulmonary arteriovenous malformations and patients with hereditary hemorrhagic telangiectasia (HHT) about their diagnosis and its clinical implications, complications, and hereditary nature.

Miscellaneous

Medicolegal Pitfalls

  • Percutaneous needle biopsy of a solitary pulmonary nodule may lead to catastrophic pulmonary hemorrhage because of a pulmonary arteriovenous malformation; therefore, consider pulmonary arteriovenous malformations in the differential diagnosis of a pulmonary nodule and rule out pulmonary arteriovenous malformations before performing needle biopsy.
  • Obtain a chest radiograph to rule out pulmonary arteriovenous malformations in patients presenting with a brain abscess.
  • The family members of patients with hereditary hemorrhagic telangiectasia (HHT) or pulmonary arteriovenous malformations should be screened with the methods described in Special Concerns below.
  • In all patients, consider intrapulmonary shunts as causes of hypoxemia because of pulmonary arteriovenous malformations.

Special Concerns

  • Screening of family members with pulmonary arteriovenous malformations or HHT may be helpful.
    • Family members of patients with HHT should be routinely screened for possible pulmonary arteriovenous malformations.
    • The incidence of pulmonary arteriovenous malformations is approximately 15-20% in unselected patients with HHT.
    • Screening families with HHT is particularly important when pulmonary arteriovenous malformation has already been diagnosed in at least one relative.
    • The incidence of pulmonary arteriovenous malformations is approximately 35% among relatives of persons HHT.
  • Evaluate all patients by obtaining a history and by performing a focused physical examination, chest radiography, and a 100% oxygen shunt study.
    • Patients with an increased shunt fraction may be referred for contrast CT scanning or pulmonary angiography, whereas patients with abnormal chest radiographic findings combined with a shunt fraction in the reference range are referred for contrast echocardiography, followed by angiography if the echocardiographic findings are normal.
    • Routine screening of family members with CT or contrast echocardiography is expensive, and pulse oximetry is insensitive as a screening tool.
    • Routine genetic screening of family members of patients with HHT is likely to play an increasingly important role in the future. The current screening methods for endoglin mutations and mutations in the active ALK-1 gene are useful in to detecting responsible mutations in 75-100% of families with HHT. Once the genetic defect is identified for a given family with HHT, genetic screening is 100% sensitive and specific in determining the presence of clinical HHT in individual family members. Therefore, in families with a known genetic defect, genetic screening of all family members by analyzing their peripheral blood is recommended.
  • Pregnancy
    • The risks in pregnant women with HHT and pulmonary arteriovenous malformations are significant, and knowledge of these risks is important in counseling of affected individuals and families.
    • In one series, maternal complications occurred in almost half of pregnancies involving patients with pulmonary arteriovenous malformations. In pregnant women with pulmonary arteriovenous malformations, worsening of right-to-left shunt, fatal pulmonary hemorrhage, and stroke occurred. Progression of vascular malformations in the cerebral, pulmonary, and hepatic circulation during pregnancy has also been documented. Therefore, all women with HHT or a family history of HHT who are considering pregnancy should be screened for pulmonary arteriovenous malformations.
  • Acquired pulmonary arteriovenous malformations after surgery for congenital cyanotic heart disease
    • The Glenn procedure is superior vena cava (SVC)-to-right pulmonary artery (RPA) anastomosis; this has been used to bypass tricuspid atresia in congenital heart diseases. The Fontan procedure creates a communication from the right atrium to the pulmonary artery, and one of its early modifications interposes a communication from the right atrium to the right ventricle. Both of these procedures are associated with the development of intrapulmonary shunting via the pulmonary arteriovenous malformations approximately 6-10 years after the repair procedure.
    • Although microscopic pulmonary-to-systemic arterial shunting is observed in most patients, important pulmonary arteriovenous malformations confirmed by 2-dimensional echocardiographic contrast study have been reported in 25% of the patients.13,14 The currently popular hemi-Fontan (side-to-side SVC-to-RPA anastomosis) operation and, to a lesser extent, the fenestrated lateral tunnel Fontan operation are also associated with the development of pulmonary arteriovenous malformations.
    • The mechanism of pulmonary arteriovenous malformation is hypothesized to be multifactorial and involves low pulsatile pulmonary blood flow, predominant perfusion to the lower lobes, and diminished flow to the upper and middle lobes exacerbated by gravitational effects. Hypoxemia and hemoconcentration may be other factors contributing to the formation of pulmonary arteriovenous malformations.
    • The development of pulmonary arteriovenous malformations is clinically suspected when cyanosis, hypoxemia, and dyspnea return. The presence of pulmonary arteriovenous malformations is confirmed by means of radionuclide scanning or 2-dimensional echocardiographic contrast study. To determine the hemodynamic significance of pulmonary arteriovenous malformations and to identify their exact locations, further evaluation by means of cardiac catheterization and pulmonary angiography may be undertaken.15
    • Treatment of a significant pulmonary arteriovenous malformation includes therapeutic embolization or ligation of venous collaterals and operative reconstruction. Percutaneous transcatheter embolization is the least invasive technique and generally consists of embolization with coils or balloons. The procedure may be repeated at a later date if a significant shunt still persists. The adverse effects of embolization are pulmonary infarction, chest pain, fever, and pleural effusions. Reconstruction of SVC-to–right atrium continuity and RPA ligation have been suggested. Reimplantation of the pulmonary veins of the right lower lobe to the right atrium has also been recommended. These procedures may be associated with significant morbidity or mortality rates.16
 


More on Arteriovenous Fistulae, Pulmonary

Overview: Arteriovenous Fistulae, Pulmonary
Differential Diagnoses & Workup: Arteriovenous Fistulae, Pulmonary
Treatment & Medication: Arteriovenous Fistulae, Pulmonary
Follow-up: Arteriovenous Fistulae, Pulmonary
Multimedia: Arteriovenous Fistulae, Pulmonary
References
Further Reading
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References

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Further Reading

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Keywords

pulmonary arteriovenous fistulae, pulmonary arteriovenous malformation, PAVM, pulmonary AVM, pulmonary arteriovenous fistula, Rendu-Osler-Weber syndrome, Rendu-Osler-Weber disease, Osler disease, Osler's disease, telangiectasia, hereditary hemorrhagic telangiectasia, HHT, arteriovenous malformation, AVM, cirrhosis, schistosomiasis, mitral stenosis, actinomycosis, metastatic thyroid carcinoma, bronchiectasis, cerebrovascular malformations, stroke, brain abscess, iron-deficiency anemia, Fanconi syndrome, diagnosis, treatment

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Contributor Information and Disclosures

Author

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Heart Rhythm Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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