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A 46-Year-Old Woman With Left Shoulder Pain and Worsening DyspneaLeft Shoulder Pain and Worsening Dyspnea FREE TO VIEW

Shuang Liu, MD; Xiaoyun Li, MD; Xiaoyong Huang, MD; Tiezheng Li, MD; Ya Yang, MD; Renli Qiao, MD, PhD, FCCP
Author and Funding Information

From the Department of Pulmonary and Critical Care Medicine (Drs Liu and X. Li), the Department of Interventional Radiology (Drs Huang and T. Li), and the Department of Ultrasound (Dr Yang), Beijing Anzhen Hospital, The Capital Medical University, Beijing, China; and the Department of Pulmonary and Critical Care Medicine (Dr Qiao), University of Southern California, Los Angeles, Los Angeles, CA.

CORRESPONDENCE TO: Shuang Liu, MD, Department of Pulmonary and Critical Care Medicine, No. 2 Anzhen Rd, Chaoyang District Beijing Anzhen Hospital, Beijing 100029, China; e-mail: liushuang862002@163.com


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;146(3):e84-e87. doi:10.1378/chest.14-0251
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A 46-year-old woman presented with worsening dyspnea and palpitations for 3 days following an episode of left shoulder pain. The patient had received a diagnosis of patent ductus arteriosus with Eisenmenger syndrome in childhood but had been inconsistently taking digoxin and diuretics since then. She was married but had never been pregnant. Over the previous 2 to 3 years, her exercise capacity had decreased significantly, with frequent episodes of near syncope and chest pain. She had developed bilateral leg edema. Three days prior to admission, she noticed an episode of severe, sharp pain in her left shoulder that was nonradiating but associated with palpitations and sweating and that lasted for about 20 min. The shoulder pain subsided spontaneously but her dyspnea worsened significantly, which prompted her to present to the ED.

Physical Examination Findings

On physical examination, her temperature was 36.5°C, respiratory rate was 25/min, heart rate was 100 beats/min, and BP was 90/60 mm Hg. The patient was thin and appeared anxious. There was jugular vein distention. A continuous harsh murmur was best heard in the left parasternal third intercostal space, and the pulmonic component of the second heart sound was accentuated. The lungs were clear. The abdomen was distended with shifting dullness. A smooth liver edge was 3 cm below the costal margin. There was pitting edema in both legs and mild cyanosis and clubbing of the fingers.

Diagnostic Studies

The CBC count demonstrated a normal WBC count and a hematocrit value of 48%. Liver enzymes were mildly elevated. Electrolyte panel values and renal function were within the normal range. Troponin T level was 0.23 μg/L (normal, 0-0.1 μg/L). Arterial blood gas analysis on room air showed a pH of 7.44, Paco2 of 31.3 mm Hg, Pao2 of 55.4 mm Hg, and oxygen saturation of 90.3%. A chest radiograph showed an enlarged heart with prominent pulmonary vessels, but no signs of pulmonary edema or infiltrates (Fig 1). A 12-lead ECG showed sinus rhythm with an incomplete right bundle branch block and a right ventricular strain pattern with no ischemic changes. A two-dimensional transthoracic echocardiogram showed a dilated, severely hypokinetic right ventricle with flattening of the interventricular septum, suggestive of right ventricular pressure overload. The right atrium was dilated with tricuspid regurgitation, and the estimated pulmonary artery systolic pressure was 69 mm Hg. Additional findings are shown in Fig 2.

Figure Jump LinkFigure 1  Chest radiograph shows an enlarged heart with prominent pulmonary arteries. No sign of pulmonary edema or infiltrates.Grahic Jump Location
Figure Jump LinkFigure 2  Echocardiography. A, Four-chamber view. B, Focused view of the PA. The arrows point to the flap. F = false lumen; LA = left atrium; LV = left ventricle; PA = pulmonary artery; RA = right atrium; RV = right ventricle; T = true lumen.Grahic Jump Location
What is the diagnosis?
Diagnosis: Pulmonary artery dissection

Despite recent progress in pharmacologic treatment, severe pulmonary hypertension, regardless of cause, is still associated with a high mortality. However, the cause of death in patients with pulmonary hypertension is surprisingly unclear. In a recent study specifically addressing the topic, 44% of deaths in patients with group 1 pulmonary hypertension were “directly related to PH” with the specific cause of death listed as right-sided heart failure/sudden death. Exactly what the possible mechanisms were for sudden death was not explored further. Although the extreme hypertrophy of the right ventricle from severe pulmonary hypertension could result in right ventricular ischemia/infarction and arrhythmia, pulmonary artery dissection is another consideration.

Pulmonary artery dissection is an extremely rare event and used to be a diagnosis established at autopsy. The majority of reported cases are young patients with chronic pulmonary hypertension secondary to congenital heart abnormalities, most commonly patent ductus arteriosus (PDA). The dissection is caused by pathologic changes such as medial necrosis in the vascular wall, presumably from mechanical shear stress or inflammatory damage. Progress in imaging technology has made it possible to detect pulmonary artery dissection during life. The main pulmonary trunk is almost exclusively the site of dissection, especially if Eisenmenger syndrome has developed. The clinical presentation is nonspecific, but sudden onset of chest pain in a patient with severe pulmonary hypertension should prompt careful transthoracic echocardiogram examination. An extremely dilated pulmonary artery (probably > 6 cm) should trigger a careful search for the presence of a flap or any sign of disruption in the vascular wall. If the echocardiogram raises any suspicion, either a CT scan or a magnetic resonance (MR) pulmonary angiogram should be obtained. The multiplanar reformation and three-dimensional reconstruction capabilities of modern imaging technologies can delineate the dissection flap in great detail, as seen in the current case.

Although isolated success with conservative management has been reported, the majority of survivors with pulmonary artery dissection underwent surgical interventions. However, when a pulmonary dissection is associated with severe pulmonary hypertension and congenital heart disease, the surgical decision can become extremely difficult because the severe pulmonary hypertension and Eisenmenger syndrome are considered to be contraindications for surgical repair, especially for closure of the PDA. Under such circumstances, careful evaluation is recommended before surgery is attempted. If severe pulmonary arterial hypertension is present, corrective surgery of shunts should be considered only for patients with evidence of pulmonary arterial reactivity, the presence of left-to-right shunting of at least 1.5 to 1.0, or both. However, even the pulmonary arterial catheterization must be performed with extreme caution because of the risk of rupture of the dissected pulmonary artery.

Clinical Course

As seen in Fig 2, the echocardiogram demonstrated a severely enlarged pulmonary artery of > 6 cm (Fig 2A) and a flap in the main pulmonary trunk separating the vessel into true and false lumens (Fig 2B), communicating through an opening of 10.6 mm. Color Doppler flow imaging showed a maximal velocity of 131 cm/s in the true lumen and 79 cm/s in the false lumen. A large PDA was seen between the descending aorta and the pulmonary artery bifurcation (width of 8.2 mm) with right-to-left shunting. MR pulmonary angiography was obtained, which confirmed the enlargement of the main pulmonary artery trunk, in which the linear dissection was seen to extend from the right pulmonary valve to the origin of the right pulmonary artery (Fig 3). Surgical repair was considered, but the best approach was unclear because a significant Eisenmenger shunt would be a contraindication for surgical intervention. After discussion among teams and with the family, a cardiac catheterization was performed. The pulmonary arterial pressure was 107/51 mm Hg, and pulmonary vascular resistance was 384.32 dynes/s/cm5. The right-to-left shunt was confirmed with a shunt fraction estimated to be > 30%. Three days after the right-sided heart catheterization, although a surgical plan was still being discussed, the patient suddenly went into pulseless electrical activity and could not be resuscitated. The family declined autopsy, and the presumed cause of death was pulmonary arterial rupture.

Figure Jump LinkFigure 3  Magnetic resonance pulmonary angiography showing the dilated main pulmonary artery. A linear dissection with a flap (arrow in both A and B) is demonstrated. A, axial view. B, sagittal view.Grahic Jump Location

  • 1. Pulmonary artery dissection is a complication of severe pulmonary hypertension and a possible cause of sudden death.

  • 2. Clinical presentation is nonspecific but chest pain in the setting of severe pulmonary hypertension should raise the suspicion.

  • 3. Echocardiography can provide initial diagnostic clues; CT scan or MR pulmonary angiography can delineate the dissection and guide management.

  • 4. Surgical repair can prevent rupture but requires careful preoperative evaluation to determine the risks and benefits of the appropriate approach.

  • 5. Extreme caution is necessary if catheterization of the dissected pulmonary artery is considered.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Walley VM, Virmani R, Silver MD. Pulmonary arterial dissections and ruptures: to be considered in patients with pulmonary arterial hypertension presenting with cardiogenic shock or sudden death. Pathology. 1990;22(1):1-4. [CrossRef] [PubMed]
 
Tønder N, Køber L, Hassager C. Pulmonary artery dissection in a patient with Eisenmenger syndrome treated with heart and lung transplantation. Eur J Echocardiogr. 2004;5(3):228-230. [CrossRef] [PubMed]
 
Khush KK, Randhawa R, Israel E. A full house: complications from an uncorrected patent ductus arteriosus. Curr Cardiol Rep. 2005;7(4):310-313. [CrossRef] [PubMed]
 
Aldrovandi A, Monti L, Corrada E, Profili M, Presbitero P. Eisenmenger syndrome complicated by pulmonary artery dissection. Eur Heart J. 2007;28(3):298. [CrossRef] [PubMed]
 
Westaby S, Evans BJ, Ormerod O. Pulmonary-artery dissection in patients with Eisenmenger’s syndrome. N Engl J Med. 2007;356(20):2110-2112. [CrossRef] [PubMed]
 
Ay Y, Ay NK, Aydin C, Kara I, Zeybek R. A rare complication of pre-Eisenmenger patent ductus arteriosus: pulmonary artery dissection. Int J Surg Case Rep. 2013;4(5):483-485. [CrossRef] [PubMed]
 
Tonelli AR, Arelli V, Minai OA, et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2013;188(3):365-369. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1  Chest radiograph shows an enlarged heart with prominent pulmonary arteries. No sign of pulmonary edema or infiltrates.Grahic Jump Location
Figure Jump LinkFigure 2  Echocardiography. A, Four-chamber view. B, Focused view of the PA. The arrows point to the flap. F = false lumen; LA = left atrium; LV = left ventricle; PA = pulmonary artery; RA = right atrium; RV = right ventricle; T = true lumen.Grahic Jump Location
Figure Jump LinkFigure 3  Magnetic resonance pulmonary angiography showing the dilated main pulmonary artery. A linear dissection with a flap (arrow in both A and B) is demonstrated. A, axial view. B, sagittal view.Grahic Jump Location

Tables

Suggested Readings

Walley VM, Virmani R, Silver MD. Pulmonary arterial dissections and ruptures: to be considered in patients with pulmonary arterial hypertension presenting with cardiogenic shock or sudden death. Pathology. 1990;22(1):1-4. [CrossRef] [PubMed]
 
Tønder N, Køber L, Hassager C. Pulmonary artery dissection in a patient with Eisenmenger syndrome treated with heart and lung transplantation. Eur J Echocardiogr. 2004;5(3):228-230. [CrossRef] [PubMed]
 
Khush KK, Randhawa R, Israel E. A full house: complications from an uncorrected patent ductus arteriosus. Curr Cardiol Rep. 2005;7(4):310-313. [CrossRef] [PubMed]
 
Aldrovandi A, Monti L, Corrada E, Profili M, Presbitero P. Eisenmenger syndrome complicated by pulmonary artery dissection. Eur Heart J. 2007;28(3):298. [CrossRef] [PubMed]
 
Westaby S, Evans BJ, Ormerod O. Pulmonary-artery dissection in patients with Eisenmenger’s syndrome. N Engl J Med. 2007;356(20):2110-2112. [CrossRef] [PubMed]
 
Ay Y, Ay NK, Aydin C, Kara I, Zeybek R. A rare complication of pre-Eisenmenger patent ductus arteriosus: pulmonary artery dissection. Int J Surg Case Rep. 2013;4(5):483-485. [CrossRef] [PubMed]
 
Tonelli AR, Arelli V, Minai OA, et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2013;188(3):365-369. [CrossRef] [PubMed]
 
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