Robert I. White

Percutaneous Balloon Valvuloplasty: A New Method for Treating Congenital Pulmonary-Valve Stenosis

TRANSLUMINAL balloon angioplasty has been increasingly accepted as a nonsurgical technique for dilatation of stenotic arteries in the peripheral, renal, and coronary circulations.1 2 3 4 5 6 The ph...

International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia

Background HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. Objective The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. Methods The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. Results The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

Liver Disease in Patients with Hereditary Hemorrhagic Telangiectasia

BACKGROUND Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber disease, is an autosomal dominant disorder characterized by angiodysplastic lesions (telangiectases and arteriovenous malformations) that affect many organs. Liver involvement in patients with this disease has not been fully characterized. METHODS We studied the clinical findings and results of hemodynamic, angiographic, and imaging studies in 19 patients with hereditary hemorrhagic telangiectasia and symptomatic liver involvement. RESULTS We evaluated 14 women and 5 men who ranged in age from 34 to 74 years. All but one of the patients had a hyperdynamic circulation (cardiac index, 4.2 to 7.3 liters per minute per square meter of body-surface area). In eight patients, the clinical findings were consistent with the presence of high-output heart failure. The cardiac index and pulmonary-capillary wedge pressure were elevated in the six patients in whom these measurements were performed. After a median period of 24 months, the condition of three of the eight patients had improved, four were in stable condition with medical therapy, and one had died. Six patients had manifestations of portal hypertension such as ascites or variceal bleeding. The hepatic sinusoidal pressure was elevated in the four patients in whom it was measured. After a median period of 19 months, the condition of two of the six patients had improved, and the other four had died. Five patients had manifestations of biliary disease, such as an elevated alkaline phosphatase level and abnormalities on bile duct imaging. After a median period of 30 months, the condition of two of the five had improved, the condition of one was unchanged, heart failure had developed in one, and one had died after an unsuccessful attempt at liver transplantation. CONCLUSIONS In patients with hereditary hemorrhagic telangiectasia and symptomatic liver-involvement, the typical clinical presentations include high-output heart failure, portal hypertension, and biliary disease.

Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy.

The recent long-term studies from England, France, and the Netherlands, as well as our own, indicate that transcatheter embolotherapy is definitive treatment for PAVM. More recently, Puskas et al have questioned transcatheter embolotherapy as a primary treatment for patients with PAVM (4,56). Their opinion was based on two recurrences among five patients treated with transcatheter embolotherapy. It is not clear why one of the late recurrences in the series by Puskas et al happened, and the other recurrence could have been dut to early deflation of the balloon. Nevertheless, we believe that the collective experience in the larger series reporting on transcatheter embolotherapy of PAVM supports the use of embolotherapy as a primary modality of treatment. Because many patients have bilateral pulmonary malformations and many pulmonary malformations will grow with time, repeated surgical intervention is not ideal therapy. The recurrence rate of 8% reported by Remy et al using coils, and 2% reported by Pollak et al using balloons and coils supports our contention that transcatheter embolotherapy is durable and should be the initial treatment. Also, recurrences are easily retreated by transcatheter embolotherapy with durable results (54). We favor detachable balloons over coils for occluding PAVMs because immediate cross-sectional occlusion of the segmental artery is obtained in a position that preserves the most normal branches. The necessity for repeated introduction of coils, when using the coil method, contributes to longer procedure times with an increased risk of air introduction and, in our experience, a greater risk of postprocedure pleurisy. At the same time, we appreciate that approximately 70% of PAVMs can be occluded equally well with balloons or coils. We also believe that coils have unique advantages over balloons in specific anatomic situations including oversized arteries (where coils are the only option) and for occlusion of the aneurysm of a PAVM. As with all forms of embolotherapy, the interventionalist is best served by having more than one option of treatment, which for PAVM includes both balloons and coils. In summary, PAVMs are effectively managed by means of transcatheter embolotherapy. This therapy has been demonstrated to be safe and durable. Careful technique with modifications depending on the angioarchitecture of the PAVM is required. Patients with PAVMs require follow-up at 1 month and 1 year. While observations documenting serial growth of small PAVMs are somewhat limited, there is published evidence to support their growth with time (35,36). Because of these reports and our unpublished observations, we believe that patients with treated PAVM need long-term follow-up every 5 years to detect growth of small PAVMs that will ultimately reach a size where they may cause paradoxical embolization and stroke (1).

Embolotherapy of large pulmonary arteriovenous malformations: long-term results.

BACKGROUND The purpose of this study was to document the long-term results of transcatheter embolotherapy of large pulmonary arteriovenous malformations (PAVMs). METHODS From a data base of 221 consecutive patients with PAVMs treated by embolotherapy between 1978 and 1995, 45 patients with 52 PAVMs, supplied by feeding arteries 8 mm in diameter or larger, were selected for a retrospective investigation. RESULTS Of 45 patients with 52 large PAVMs, 38 patients (84%) with 44 PAVMs (85%) were cured by the first embolotherapy (mean follow-up, 4.7 years). Acute periprocedural complications included self-limited pleurisy (31%), angina secondary to air embolus (2%), and paradoxical embolization of a device during deployment (4%). None of these events led to short- or long-term sequelae. Seven patients (16%) had persistence of the PAVM attributable to either recanalization (n = 4) or interim accessory artery growth (n = 3). Two of these patients presented with ischemic stroke several years after the initial treatment. Persistent PAVMs (n = 8) were retreated successfully by a second procedure (n = 7), or a third procedure (n = 1) (mean follow-up, 5.9 and 5.3 years, respectively). CONCLUSIONS Embolotherapy of large PAVMs results in permanent occlusion in an overwhelming majority of patients. Continued patency due to recanalization or accessory artery growth is easily detected and treated.

Pulmonary arteriovenous malformations Cerebral ischemia and neurologic manifestations

Background: There is an increasingly recognized association between pulmonary arteriovenous malformations (PAVM) and cerebral ischemia, frequently attributed to paradoxical embolization. PAVM occur in 20 to 30% of the hereditary hemorrhagic telangiectasia (HHT) population. Objective: To evaluate the risk determinants for cerebral ischemia and neurologic manifestations in patients with PAVM. Methods: A retrospective cross-sectional study was performed on consecutive patients admitted between 1988 and 1992 for treatment of PAVM. The number of PAVM, feeding artery (FA) diameters, and aneurysmal sizes were determined by pulmonary angiography. Patients were categorized as having single or multiple PAVM with an FA diameter of ≥3 mm. History, examination, and cerebral imaging studies were used to determine the prevalence of neurologic manifestations. Patients were defined as having cerebral paradoxical embolization if there was radiologic evidence of cortical infarction. Results: There were 75 cases: 26 single PAVM and 49 multiple PAVM. Cortical infarction was present in 14% of patients with single PAVM. Patients with multiple PAVM had a greater prevalence of any infarction (OR 3.2; 95% CI, 1.2 to 9.44, p = 0.030), cortical infarctions (OR 2.3; 95% CI, 0.58 to 9.2, p = 0.230), subcortical infarctions (OR 2.1; 95% CI, 0.58 to 7.95, p = 0.249), abscesses (OR 2.3; 95% CI, 0.46 to 11.94; p = 0.295), and seizures (OR 6.4, 95% CI 0.77 to 53.2, p = 0.054). Patients with multiple PAVM had markedly greater odds of having any clinical or radiologic evidence of cerebral ischemic involvement (OR 4.5; 95% CI, 1.47 to 14; p = 0.008). Conclusion: There is a strong association between single PAVM and various neurologic manifestations. The prevalence is greater for patients with multiple PAVM, suggesting increased predisposition for paradoxical embolization with a greater number of malformations.

Percutaneous transluminal balloon valvuloplasty for pulmonary valve stenosis.

Transluminal balloon valvuloplasty was used to treat congenital pulmonary valve stenosis in 20 patients. Follow-up cardiac catheterization was performed in 11 patients at intervals of from 2 to 12 months after the procedure. Peak systolic pressure gradient across the pulmonic valve decreased from 68 +/- 27 to 23 +/- 5 mm Hg (p less than .001) after valvuloplasty. There were no complications. Follow-up catheterization demonstrated persistent relief of right ventricular hypertension in the patients with typical pulmonary valve stenosis.

Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations

PURPOSE To assess long-term clinical and imaging results of technically successful pulmonary arteriovenous malformation (AVM) embolization. MATERIALS AND METHODS One hundred fifty-five patients with pulmonary AVMs underwent embolization during a period of 3 years. Recommended follow-up included clinical assessment, helical computed tomography, and physiologic evaluation within 1 year and then every 5 years. RESULTS Hereditary hemorrhagic telangiectasia was present in 148 patients (95%). Four hundred fifteen pulmonary AVMs were occluded during 205 procedures. Clinical follow-up was available in all patients over 3-7 years and imaging follow-up was available in 144 patients (393 lesions) over 1-7 years (mean, 2.9 y). Problems related to pulmonary AVMs occurred in 35 patients (23%) at 42 time points: 22 patients with 23 symptomatic events and 17 patients with 19 asymptomatic events. Symptoms resulted from growth of nonembolized pulmonary AVMs (n = 19), residual embolized pulmonary AVMs (n = 5), or both (n = 2). Symptoms consisted of respiratory manifestations (n = 13), cerebral ischemia (n = 4), brain abscess (n = 5), hemoptysis (n = 3), and seizure (n = 1). Imaging showed pulmonary AVM involution in 97% of embolized lesions and 11 residual lesions (2.8%) in 10 patients (6.9%). These were caused by recanalization (n = 7), presence of an accessory feeding artery (n = 1), pulmonary collateral vessels (n = 1), and bronchial collateral vessels (n = 2). CT detected 10 of the 11 residual lesions. Imaging detected 97 previously small pulmonary AVMs that had enlarged to a significant size in 28 patients (18%), 15 of whom were symptomatic and 13 of whom were asymptomatic. CONCLUSIONS Clinical and anatomic evaluation after pulmonary AVM embolization is important to detect persistent or reperfused lesions and enlarging lesions, with the latter more common. Patients with persistent, reperfused, or enlarging lesions often have symptoms, but a significant minority of patients are asymptomatic. More frequent assessment may improve detection before the onset of symptoms.

Benign postoperative biliary strictures. Operate or dilate

At The Johns Hopkins Hospital from 1979 through 1987, 42 patients had 45 procedures for benign postoperative biliary strictures. Three patients were managed with both surgery and balloon dilatation. Twenty-five patients underwent surgical repair with Roux-Y choledocho- or hepaticojejunostomy with postoperative transhepatic stenting for a mean of 13.8 +/- 1.3 months. Twenty patients had balloon dilatation a mean of 3.9 times and were stented transhepatically for a mean of 13.3 +/- 2.0 months. The two groups were similar with respect to multiple parameters that might have influenced outcome. Mean length of follow-up was 57 +/- 7 and 59 +/- 6 months for surgery and balloon dilatation, respectively. No patients died after any of the procedures. The same definition of a successful outcome was applied to both groups and was achieved in 88% of the surgical and in only 55% of the balloon dilatation patients (p less than 0.02). Significant hemobilia occurred more often with balloon dilatation (20% vs. 4%, p less than 0.02). The total hospital stay and cost of balloon dilatation was not significantly different from surgery. We conclude that surgical repair of benign postoperative strictures results in fewer problems that require further therapy. Nevertheless balloon dilatation is an alternative for patients who are at high risk or who are unwilling to undergo another operation.

The Use of Cyanoacrylate Adhesives in Peripheral Embolization

Although liquid adhesives or glue have been used as embolic agents for nearly three decades, experience with them outside of neurointerventional indications is generally limited. Cyanoacrylates are the main liquid adhesives used in the vascular system and have an important role in managing vascular abnormalities, especially arteriovenous malformations. Vascular occlusion results as these agents polymerize on exposure to the ions in blood. A description of the properties, biologic interactions, techniques of use, and indications for acrylic embolization in the peripheral circulation is especially pertinent at this time because of the recent approval of n-butyl cyanoacrylate by the United States Food and Drug Administration.