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Sugarman syndrome

Sugarman syndrome is the common name of Oral-facial-digital syndrome type III

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Acute Massive Pulmonary Embolism in a Jehovah's Witness - )
From CHEST, 2/1/00 by Howard S. Blaustein

Successful Treatment With Catheter Thrombectomy

A 71-year-old woman presented with an acute, massive pulmonary embolism. As a Jehovah's Witness, she was not willing to accept thrombolysis because of the potential risk of bleeding requiring blood transfusion. The patient was successfully treated with catheter thrombectomy, using rheolytic and fragmentation devices.

(CHEST 2000; 117:594-597)

Key words: catheter thrombectomy; Jehovah's Witness; pulmonary embolism; thrombolysis

Abbreviation: PE = pulmonary embolism

Acute massive pulmonary embolism (PE) accounts for approximately 50,000 deaths per year in the United States.[1] The options for treatment of massive PE, in addition to the standard treatment of anticoagulation with heparin, include the following: thrombolytic therapy, open surgical embolectomy, and catheter thrombectomy.[2] Intracranial hemorrhage after thrombolysis for PE is an infrequent but grave complication.[3] In addition to specific contraindications to thrombolytic therapy, a patient may be unwilling to accept the potential risk of bleeding that would require the transfusion of blood products. With recent technologic advances in thrombectomy devices, catheter thrombectomy may be an option. We report a Jehovah's Witness patient with an acute massive PE who was successfully treated with catheter thrombectomy.


The patient is a 71-year-old African-American woman with a history of poorly controlled arterial hypertension. She presented with the acute onset of shortness of breath at rest, worsening over 4 days. There was no cough, hemoptysis, fever, or chest pain. She denied any history of smoking, cancer, or immobilization.

On admission, her BP was 130/108 mm Hg, pulse rate was 148 beats/min, and respiration rate was 26 breaths/min. The examination was significant for a right ventricular S3 gallop, and left basilar rales. The chest radiograph showed a small left pleural effusion and oligemia of the right upper lung field. An arterial blood gas analysis, drawn while breathing room air, showed pH 7.46, Pa[CO.sub.2] of 27 mm Hg, and Pa[O.sub.2] of 50 mm Hg. ECG revealed sinus tachycardia, left ventricular hypertrophy, and lateral ST-segment depression. An emergency transthoracic echocardiogram in the emergency department was technically limited, and showed normal left ventricular function and normal right ventricular size. The patient was started on IV heparin for suspected PE. The oxygen saturation was 95% while breathing 100% oxygen. A ventilation/perfusion scan (Fig 1) showed absent perfusion of the right lung with normal ventilation, consistent with a high probability of PE.


Over the next 3 days, the patient remained tachypneic, with desaturation with movement and persistence of the S3 gallop. The BP remained at 120/80 mm Hg. Thrombolysis was discussed with the patient, and the risk of bleeding and the potential requirement for blood product transfusion was explained. As a practicing Jehovah's Witness, she refused thrombolysis because of this risk of bleeding and transfusion.

Because of the severe compromise from massive PE and the perfusion scan suggesting a central right pulmonary artery thrombus, the patient was referred to the interventional radiologist for an attempt at catheter thrombectomy. Pulmonary angiography revealed thrombus completely occluding the right main pulmonary artery and the mean pulmonary artery pressure to be 30 mm Hg. A catheter thrombectomy was performed using rheolytic and fragmentation devices. A 10F Arrow flexible sheath (Arrow International; Reading, PA) was placed in the right pulmonary artery. A 0.018-inch wire was advanced into multiple thrombosed distal basal segments followed by the 5F 105-cm thrombectomy system (Angiojet; Possis; Minneapolis, MN). The system was activated for 2 to 3 min in segments of the lower and middle lobes, and in one upper lobe segment. The Possis system was then replaced with an 8F 120-cm Amplatz thrombectomy device (Microvena; White Bear Lake, MN). The device was activated in the right main pulmonary artery and proximal interlobar and upper lobe arteries for approximately 6 min.

There was definitive angiographic improvement in the perfusion of the right upper lobe and right middle lobe vessels, with restoration of approximately 60% of the right pulmonary perfusion. An inferior vend vena filter was placed.

The following morning, the patient appeared less dyspneic and the S3 gallop was no longer auscultated. A lower-extremity duplex Doppler ultrasound examination was negative for thrombus.

A ventilation/perfusion scan 2 days postthrombectomy showed reperfusion of the right upper and middle lobes (Fig 2). Perfusion was further improved on scanning 2 weeks later. The patient was discharged on the 17th hospital day receiving warfarin anticoagulation. The patient is doing well, without dyspnea, 4 months later.



Thrombolysis is indicated for circulatory and respiratory failure from massive PE. However, thrombolysis may be complicated by bleeding, with infrequent but often fatal intracranial hemorrhage.[3] For patients at risk of bleeding or unwilling to accept the risk of hemorrhage or transfusion, catheter thrombectomy may be an alternative emergency treatment.

There are currently three catheter thrombectomy techniques: aspiration thrombectomy, fragmentation thrombectomy, and rheolytic thrombectomy.[4,5] The first percutaneous catheter thrombectomy device, an aspiration catheter, was introduced in 1969 by Greenfield et al[6]; in 1993, he reported his long-term experience showing a success rate of 82% for massive PE.[2] In 1997, a rheolytic thrombectomy, in which high-pressure saline solution jets at the tip of the catheter lyse the thrombi (which are then aspirated), was successfully used in two patients with severe PE.[5]

Uflacker et al[7] reported the use in five patients of a fragmentation technique, the Amplatz thrombectomy device, in which an impeller creates a vortex that pulverizes the thrombus. There was marked improvement in perfusion in one patient and moderate improvement in three patients. In a series reported by Schmitz-Rode et al,[8] a pigtail rotation catheter was successful in 7 of 10 patients with acute massive PE.

Reported complications of catheter thrombectomy include pulmonary infarction, wound hematoma and infection, myocardial infarction, and ventricular perforation.[2] A reperfusion syndrome with hemorrhage and focal pulmonary edema has been described.[7]

In our ease, an acute massive PE in a Jehovah's Witness was successfully treated with catheter thrombectomy. The use of two catheter thrombectomy techniques, rheolytic and fragmentation, during one procedure was unique. The patient's previously untreated arterial hypertension was a risk for an intracranial hemorrhage; however, the BP was in the normal range during the critical care unit course. As a Jehovah's Witness, she was not willing to undergo a transfusion of any blood products in the event of bleeding at any site.

Jehovah's Witnesses hold religious beliefs that preclude accepting blood products, although this issue must be addressed with each individual patient. Major surgery and management of bleeding may be accomplished without transfusion[9]; however, offering thrombolytic therapy raises other issues.

The scope of this problem in patients receiving thrombolytic therapy for acute myocardial infarction has not been formally addressed in the literature. In 1991, Sugarman et al[10] described a patient who was given thrombolysis for myocardial infarction and died of GI hemorrhage; the patient was a Jehovah's Witness and refused blood transfusion, despite the risk of death. The authors raised the question of whether refusal of blood products should be considered in the decision to administer thrombolytic therapy. Sugarman et al[10] specifically did not want to exclude Jehovah's Witnesses from receiving the benefit of thrombolysis, and stressed informed consent about the potential complications and potential transfusion requirement. In addition, the authors suggested that this consideration be applied to patients who refuse blood products for any reason.

In a patient with massive PE in whom thrombolysis is medically indicated and who, after informed consent, refuses thrombolysis and potential transfusion, catheter thrombectomy may be an attractive alternative.


[1] Goldhaber SZ. Contemporary pulmonary embolism thrombolysis. Chest 1995; 107(suppl):45S-51S

[2] Greenfield LJ, Proctor MC, Williams DM, et al. Long term experience with transvenous catheter pulmonary embolectomy. J Vasc Surg 1993; 18:450-458

[3] Kanter DS, Mikkola KM, Patel SR, et al. Thrombolytic therapy for pulmonary embolism: frequency of intracranial-hemorrhage and associated risk factors. Chest 1997; 111: 1241-1245

[4] Goldhaber SZ. Integration of catheter thrombectomy into our armamentarium to treat acute pulmonary embolism. Chest 1998; 114:1237-1238

[5] Koning R, Cribier A, Gerber L, et al. A new treatment for severe pulmonary embolism: percutaneous rheolytic thrombectomy. Circulation 1997; 96:2498-2500

[6] Greenfield LJ, Kimmel D, McCurdy WC. Transvenous removal of pulmonary emboli by vacuum-cup catheter technique. J Surg Res 1969; 9:347-352

[7] Uflacker R, Strange C, Vujic I. Massive pulmonary embolism: preliminary results of treatment with the Amplatz thrombectomy device. J Vasc Interv Radiol 1996; 7:519-528

[8] Schmitz-Rode T, Janssens U, Schild HH, et al. Fragmentation of massive pulmonary embolism using a pigtail rotation catheter. Chest 1998; 114:1427-1436

[9] Mann MC, Votto J, Kambe J, et al. Management of the severely anemic patient who refuses transfusion: lessons learned during the care of a Jehovah's Witness. Ann Intern Med 1992; 117:1042-1048

[10] Sugarman J, Churchill LR, Moore JK, et al. Medical, ethical and legal issues regarding thrombolytic therapy in the Jehovah's Witness. Am J Cardiol 1991; 68:1525-1529

(*) From the Division of Pulmonary and Critical Care Medicine, Department of Interventional Radiology, St. Luke's-Roosevelt Hospital Center, New York, NY.

Manuscript received May 18, 1999; revision accepted August 16, 1999.

Correspondence to: Janet M. Shapiro, MD, FCCP, Division of Pulmonary and Critical Care Medicine, St. Luke's-Roosevelt Hospital MU 316, 1111 Amsterdam Ave, New York, NY 10025

COPYRIGHT 2000 American College of Chest Physicians
COPYRIGHT 2000 Gale Group

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