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Systemic-pulmonary arteriovenous fistulae with pulmonary hypertension

Systemic-pulmonary arteriovenous fistulae with pulmonary hypertension Background: Arteriovenous (AV) fistulae is an extremely rare disease of vascular malformation that involves fistulae formation between the systemic and pulmonary AV systems. Case representation: This case report describes a rare systemic-pulmonary AV fistulae of congenital origin, accompanied by pulmonary hypertension, as determined by aortic angiography and echocardiography. Conclusion: Characteristics, diagnosis, and therapeutic approaches of this rare abnormality are explored. Abbreviations: AV = arteriovenous, BE = buffuer excess, CT = computed tomography, FAC = fractional area change, HCO = concentration of bicarbonate ion, NT-pro BNP = N-terminal pro-brain natriuretic peptide, PCO = partial pressure of carbon dioxide, pH = pouvoir hydrogene, PO = partial pressure of oxygen, SpO = blood oxygen saturation, TAPSE = tricuspid annular plane 2 2 systolic excursion, Tei index = right ventricular index of myocardial performance. Keywords: angiography, arteriovenous fistulae, echocardiography, pulmonary hypertension 1. Introduction Department of Respiration at Beijing Chaoyang Hospital (Beijing, China) on April 20, 2015. Medical history was Connections between the systemic and pulmonary systems are remarkable for giant cell tumor of the bone 15 years before, [1,2] rare and may be congenital or acquired. Patients most often followed by replacement of the left femoral head 14 years before are asymptomatic but may show cyanosis, dyspnea, or due to tumor progression. He reported a diagnosis of gout 7 years [3] hemoptysis. Systemic artery angiography is the gold standard before. Routine physical examination on the day of admission for diagnosis, with surgery and interventional embolization being revealed elevations in body temperature (38.3°C), heart rate (120 used as the primary treatment methods. Here, we report a patient beats per minute), and respiratory frequency (30times/min). He with dyspnea who later received an unexpected diagnosis of did not appear to have cyanosis or clubbing. Routine blood, systemic-pulmonary arteriovenous (AV) fistulae of congenital biochemistry, urine, and blood coagulation tests revealed no origin. Due to the patient’s extensive lesions, his pulmonary remarkable findings. Arterial blood gas analysis showed that the pressure did not decrease to normal levels either immediately or patient had type I respiratory failure (pH 7.474, partial pressure by 1 year after treatment. Written informed consent for this case of carbon dioxide [PCO ] 24.7 mm Hg, partial pressure of report was obtained from the patient. oxygen [PO ] 53.9 mm Hg, blood oxygen saturation [SpO ] 2 2 90.2%, concentration of bicarbonate ion [HCO ] 18.4mmol/L, 2. Case report and buffer excess [BE] 2.9mmol/L). His N-terminal pro-brain natriuretic peptide (NT-pro BNP) level was 593pg/mL on the The study was approved by the ethical committee of Beijing second day after hospital admission. Chaoyang Hospital, Capital Medical University, China. Written Two days after admission, a pulmonary high-resolution informed consent was obtained. computed tomography (CT) scan revealed a small amount of A 32-year-old man presented with illness-related fever and inflammation in the lower lobes of the both lungs and an abnormal unexplained dyspnea of 2 days’ duration. He was referred to the vascular mass in the middle lobe of the right lung (Fig. 1). Echocardiography indicated that the patient had pulmonary Editor: N/A. hypertension (Fig. 2A) with right heart dysfunction. Fractional The authors have no conflicts of interest to disclose. area change (FAC) of the right ventricular area was 21.8%, tricuspid a b Department of Echocardiography, Department of Respiratory, Beijing annular plane systolic excursion (TAPSE) was 18.5mm, and right Chaoyang Hospital, Capital Medical University, Beijing, P.R. China. ventricular index of myocardial performance (Tei index) was 0.93. Correspondence: Yi-Dan Li and Xiu-Zhang Lv, Department of After antiviral and antibacterial treatment, the patient’s Echocardiography, Beijing Chaoyang Hospital, Capital Medical University, Beijing temperature dropped to a normal range on April 27, 2015. 100020, China (e-mails: Yidan_li@163.com; chaoyanglxz@163.com). Although his symptoms of asthma improved, blood oxygen Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc. saturation remained lower than normal (90.4%). Over the next This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and week, several examinations were carried out, including D-dimer reproduction in any medium, provided the original work is properly cited. (0.36mg/L FEU;  0.55mg/L indicates a smaller likelihood of Medicine (2018) 97:8(e9959) pulmonary embolism), immune-related tests, right heart contrast Received: 26 December 2017 / Accepted: 30 January 2018 echocardiography, and systemic PET/CT due to pulmonary hypertension. No major abnormalities were found. http://dx.doi.org/10.1097/MD.0000000000009959 1 Shi et al. Medicine (2018) 97:8 Medicine Figure 3. CT pulmonary angiography demonstrating that compared to the left pulmonary vessels, the right pulmonary arteries, intercostal arteries, and Figure 1. High-resolution CT showing an abnormal vascular mass (red arrow; internal mammary artery were tortuous and dilated. Red arrows on the right ∼8.02.5 mm in size) in the medial segment of the middle lobe of the right lung side of the ascending aorta point to the internal mammary artery, and near the pleura. intercostal arteries are parallel to the ribs. Area around the red arrows is the range of the fistulae. CT=computed tomography. To determine the cause of pulmonary hypertension, and accompanied by varying degrees of chest tightness, heart considering the abnormal vascular findings of the right lung, the palpitations, shortness of breath, cyanosis, and dyspnea. Lesions patient underwent imaging studies. Pulmonary ventilation/ typically are located in a unilateral lung, often in the lower lobes. perfusion imaging showed no blood flow in the right lung. Most cases of anomalous systemic arteries originate from the Pulmonary CT angiography revealed tortuous and dilated right [4–6] descending aorta. pulmonary, intercostal, internal mammary, and phrenic arteries We report a rare case of an adult patient with a wide range of (Fig. 3). Aortic angiography on May 12, 2015 led to a diagnosis fistulae, accompanied by pulmonary hypertension. The systemic of congenital multiple systemic-pulmonary AV fistulae that circulation pressure was higher than the pulmonary circulation resulted in pulmonary hypertension and hypoxia. Right cardiac pressure. Because of the multiple pulmonary arteriovenous catheterization was performed in response to the pulmonary [4] fistulae, there was systemic circulation of blood flow to the right hypertension (mean pulmonary pressure = 26 mm Hg). lung, which led to low or no normal pulmonary blood flow perfusion in the lung. Right pulmonary systems accepted the 3. Discussion abnormal arterial blood flow of the systemic arteries, which eventually resulted in pulmonary hypertension. Fistulae commu- Fistulae communication between the systemic and pulmonary nication was detected by aortic angiography. arteries or pulmonary veins is rare. According to relevant case Although systemic artery angiography is the gold standard for reports, disease onset is at a relatively young age. Most patients are asymptomatic or have hemoptysis as their first symptom, diagnosis, other ancillary tests are essential. Our patient’s Figure 2. Echocardiography with the tricuspid insufficiency method showed that the systolic blood pressure of the pulmonary artery was ∼70 mm Hg in April 2015 (A) and May 2016 (B). CT=computed tomography. 2 Shi et al. Medicine (2018) 97:8 www.md-journal.com with the right heart catheter after surgery (mean pulmonary pressure = 28 mm Hg). This outcome was thought to be a result of abnormal vascular compensation. The patient was recom- mended to have regular follow-ups, and further embolization treatment was not suggested or planned. The prognosis of congenital systemic-pulmonary AV fistulae is poor, and follow-up is important. After hospital discharge, the patient was seen and underwent echocardiography once every 6 months. Two follow-up echocardiographic examinations have been performed to date, in November 2015 and May 2016. In both cases, his pulmonary systolic pressure was ∼54 mm Hg (Fig. 2B), and his right heart function had returned to normal (FAC 39.9%, TAPSE 21.7mm, Tei index 0.47). These outcomes may be related to the patient’s age and exercise habits. In summary, the present study reports a case of rare systemic- pulmonary AV fistulae of congenital origin, accompanied by pulmonary hypertension. Various factors were assessed for their potential role in causing pulmonary hypertension in this patient. This report serves not only as an example of a rare disease in a patient with pulmonary hypertension, but also points to the importance of adequate assessment according to the patient’s unique situation. Figure 4. Aortic angiography revealed fistulae (red arrows show the range) between the internal mammary artery and pulmonary vein. References [1] Varma KK, Clarke CP. Congenital systemic-to-pulmonary arteriovenous fistula: report of a case. Aust N Z J Surg 1971;40:360–2. condition was complex. We had to rule out each potential cause [2] Wong MN, Joshi P, Sim KH. Large anomalous systemic arterial supply to the left lung without pulmonary sequestration: a rare cause of heart of pulmonary hypertension (e.g., connective tissue disorder, failure in a child. Pediatr Cardiol 2009;30:85–6. tumor, chronic thromboembolic disorder, and congenital heart [3] Khanna NN, Suparna R, Obiora A. Endovascular management of disease) through a series of comprehensive examinations. massive broncho pulmonary fistulae—a series of 28 cases. Indian Heart J Echocardiography results showing pulmonary hypertension 2014;66:S96. provided a breakthrough for the final diagnosis of the disease. [4] Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Surgical and interventional embolization therapy are effective Force for the Diagnosis and Treatment of Pulmonary Hypertension of the treatments for this kind of disease. When the lesions are limited, European Society of Cardiology (ESC) and the European Respiratory surgical resection of the diseased pulmonary lobe or ligation of Society (ERS): Endorsed by: Association for European Paediatric and the anomalous artery is an acceptable choice. However, our Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37:67–119. patient had extensive lesions and unusual systemic arteries as [5] Jariwala P, Ramesh G, Sarat Chandra K. Congenital anomalous/aberrant feeding vessels of the AV fistulae, which precluded surgical systemic artery to pulmonary venous fistula: closure with vascular plugs treatment. During aortic angiography imaging for diagnosis, we & coil embolization. Indian Heart J 2014;66:95–103. used spring coils to embolize to the patient’s right internal [6] Singhi AK, Nicholson I, Francis E, et al. Anomalous systemic arterial mammary artery (the largest abnormal artery) for treatment supply to normal basal segment of the left lung. Heart Lung Circ 2011;20:357–61. (Fig. 4). However, no change in pulmonary pressure was detected http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Medicine Wolters Kluwer Health

Systemic-pulmonary arteriovenous fistulae with pulmonary hypertension

Shi, Yan-Ping; Li, Yi-Dan; Lv, Xiu-Zhang; Yang, Yuan-Hua; NA.,
Medicine , Volume 97 (8) – Feb 1, 2018
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Wolters Kluwer Health
Copyright
Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc.
ISSN
0025-7974
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1536-5964
DOI
10.1097/MD.0000000000009959
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29465587
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Abstract

Background: Arteriovenous (AV) fistulae is an extremely rare disease of vascular malformation that involves fistulae formation between the systemic and pulmonary AV systems. Case representation: This case report describes a rare systemic-pulmonary AV fistulae of congenital origin, accompanied by pulmonary hypertension, as determined by aortic angiography and echocardiography. Conclusion: Characteristics, diagnosis, and therapeutic approaches of this rare abnormality are explored. Abbreviations: AV = arteriovenous, BE = buffuer excess, CT = computed tomography, FAC = fractional area change, HCO = concentration of bicarbonate ion, NT-pro BNP = N-terminal pro-brain natriuretic peptide, PCO = partial pressure of carbon dioxide, pH = pouvoir hydrogene, PO = partial pressure of oxygen, SpO = blood oxygen saturation, TAPSE = tricuspid annular plane 2 2 systolic excursion, Tei index = right ventricular index of myocardial performance. Keywords: angiography, arteriovenous fistulae, echocardiography, pulmonary hypertension 1. Introduction Department of Respiration at Beijing Chaoyang Hospital (Beijing, China) on April 20, 2015. Medical history was Connections between the systemic and pulmonary systems are remarkable for giant cell tumor of the bone 15 years before, [1,2] rare and may be congenital or acquired. Patients most often followed by replacement of the left femoral head 14 years before are asymptomatic but may show cyanosis, dyspnea, or due to tumor progression. He reported a diagnosis of gout 7 years [3] hemoptysis. Systemic artery angiography is the gold standard before. Routine physical examination on the day of admission for diagnosis, with surgery and interventional embolization being revealed elevations in body temperature (38.3°C), heart rate (120 used as the primary treatment methods. Here, we report a patient beats per minute), and respiratory frequency (30times/min). He with dyspnea who later received an unexpected diagnosis of did not appear to have cyanosis or clubbing. Routine blood, systemic-pulmonary arteriovenous (AV) fistulae of congenital biochemistry, urine, and blood coagulation tests revealed no origin. Due to the patient’s extensive lesions, his pulmonary remarkable findings. Arterial blood gas analysis showed that the pressure did not decrease to normal levels either immediately or patient had type I respiratory failure (pH 7.474, partial pressure by 1 year after treatment. Written informed consent for this case of carbon dioxide [PCO ] 24.7 mm Hg, partial pressure of report was obtained from the patient. oxygen [PO ] 53.9 mm Hg, blood oxygen saturation [SpO ] 2 2 90.2%, concentration of bicarbonate ion [HCO ] 18.4mmol/L, 2. Case report and buffer excess [BE] 2.9mmol/L). His N-terminal pro-brain natriuretic peptide (NT-pro BNP) level was 593pg/mL on the The study was approved by the ethical committee of Beijing second day after hospital admission. Chaoyang Hospital, Capital Medical University, China. Written Two days after admission, a pulmonary high-resolution informed consent was obtained. computed tomography (CT) scan revealed a small amount of A 32-year-old man presented with illness-related fever and inflammation in the lower lobes of the both lungs and an abnormal unexplained dyspnea of 2 days’ duration. He was referred to the vascular mass in the middle lobe of the right lung (Fig. 1). Echocardiography indicated that the patient had pulmonary Editor: N/A. hypertension (Fig. 2A) with right heart dysfunction. Fractional The authors have no conflicts of interest to disclose. area change (FAC) of the right ventricular area was 21.8%, tricuspid a b Department of Echocardiography, Department of Respiratory, Beijing annular plane systolic excursion (TAPSE) was 18.5mm, and right Chaoyang Hospital, Capital Medical University, Beijing, P.R. China. ventricular index of myocardial performance (Tei index) was 0.93. Correspondence: Yi-Dan Li and Xiu-Zhang Lv, Department of After antiviral and antibacterial treatment, the patient’s Echocardiography, Beijing Chaoyang Hospital, Capital Medical University, Beijing temperature dropped to a normal range on April 27, 2015. 100020, China (e-mails: Yidan_li@163.com; chaoyanglxz@163.com). Although his symptoms of asthma improved, blood oxygen Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc. saturation remained lower than normal (90.4%). Over the next This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and week, several examinations were carried out, including D-dimer reproduction in any medium, provided the original work is properly cited. (0.36mg/L FEU;  0.55mg/L indicates a smaller likelihood of Medicine (2018) 97:8(e9959) pulmonary embolism), immune-related tests, right heart contrast Received: 26 December 2017 / Accepted: 30 January 2018 echocardiography, and systemic PET/CT due to pulmonary hypertension. No major abnormalities were found. http://dx.doi.org/10.1097/MD.0000000000009959 1 Shi et al. Medicine (2018) 97:8 Medicine Figure 3. CT pulmonary angiography demonstrating that compared to the left pulmonary vessels, the right pulmonary arteries, intercostal arteries, and Figure 1. High-resolution CT showing an abnormal vascular mass (red arrow; internal mammary artery were tortuous and dilated. Red arrows on the right ∼8.02.5 mm in size) in the medial segment of the middle lobe of the right lung side of the ascending aorta point to the internal mammary artery, and near the pleura. intercostal arteries are parallel to the ribs. Area around the red arrows is the range of the fistulae. CT=computed tomography. To determine the cause of pulmonary hypertension, and accompanied by varying degrees of chest tightness, heart considering the abnormal vascular findings of the right lung, the palpitations, shortness of breath, cyanosis, and dyspnea. Lesions patient underwent imaging studies. Pulmonary ventilation/ typically are located in a unilateral lung, often in the lower lobes. perfusion imaging showed no blood flow in the right lung. Most cases of anomalous systemic arteries originate from the Pulmonary CT angiography revealed tortuous and dilated right [4–6] descending aorta. pulmonary, intercostal, internal mammary, and phrenic arteries We report a rare case of an adult patient with a wide range of (Fig. 3). Aortic angiography on May 12, 2015 led to a diagnosis fistulae, accompanied by pulmonary hypertension. The systemic of congenital multiple systemic-pulmonary AV fistulae that circulation pressure was higher than the pulmonary circulation resulted in pulmonary hypertension and hypoxia. Right cardiac pressure. Because of the multiple pulmonary arteriovenous catheterization was performed in response to the pulmonary [4] fistulae, there was systemic circulation of blood flow to the right hypertension (mean pulmonary pressure = 26 mm Hg). lung, which led to low or no normal pulmonary blood flow perfusion in the lung. Right pulmonary systems accepted the 3. Discussion abnormal arterial blood flow of the systemic arteries, which eventually resulted in pulmonary hypertension. Fistulae commu- Fistulae communication between the systemic and pulmonary nication was detected by aortic angiography. arteries or pulmonary veins is rare. According to relevant case Although systemic artery angiography is the gold standard for reports, disease onset is at a relatively young age. Most patients are asymptomatic or have hemoptysis as their first symptom, diagnosis, other ancillary tests are essential. Our patient’s Figure 2. Echocardiography with the tricuspid insufficiency method showed that the systolic blood pressure of the pulmonary artery was ∼70 mm Hg in April 2015 (A) and May 2016 (B). CT=computed tomography. 2 Shi et al. Medicine (2018) 97:8 www.md-journal.com with the right heart catheter after surgery (mean pulmonary pressure = 28 mm Hg). This outcome was thought to be a result of abnormal vascular compensation. The patient was recom- mended to have regular follow-ups, and further embolization treatment was not suggested or planned. The prognosis of congenital systemic-pulmonary AV fistulae is poor, and follow-up is important. After hospital discharge, the patient was seen and underwent echocardiography once every 6 months. Two follow-up echocardiographic examinations have been performed to date, in November 2015 and May 2016. In both cases, his pulmonary systolic pressure was ∼54 mm Hg (Fig. 2B), and his right heart function had returned to normal (FAC 39.9%, TAPSE 21.7mm, Tei index 0.47). These outcomes may be related to the patient’s age and exercise habits. In summary, the present study reports a case of rare systemic- pulmonary AV fistulae of congenital origin, accompanied by pulmonary hypertension. Various factors were assessed for their potential role in causing pulmonary hypertension in this patient. This report serves not only as an example of a rare disease in a patient with pulmonary hypertension, but also points to the importance of adequate assessment according to the patient’s unique situation. Figure 4. Aortic angiography revealed fistulae (red arrows show the range) between the internal mammary artery and pulmonary vein. References [1] Varma KK, Clarke CP. Congenital systemic-to-pulmonary arteriovenous fistula: report of a case. Aust N Z J Surg 1971;40:360–2. condition was complex. We had to rule out each potential cause [2] Wong MN, Joshi P, Sim KH. Large anomalous systemic arterial supply to the left lung without pulmonary sequestration: a rare cause of heart of pulmonary hypertension (e.g., connective tissue disorder, failure in a child. Pediatr Cardiol 2009;30:85–6. tumor, chronic thromboembolic disorder, and congenital heart [3] Khanna NN, Suparna R, Obiora A. Endovascular management of disease) through a series of comprehensive examinations. massive broncho pulmonary fistulae—a series of 28 cases. Indian Heart J Echocardiography results showing pulmonary hypertension 2014;66:S96. provided a breakthrough for the final diagnosis of the disease. [4] Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Surgical and interventional embolization therapy are effective Force for the Diagnosis and Treatment of Pulmonary Hypertension of the treatments for this kind of disease. When the lesions are limited, European Society of Cardiology (ESC) and the European Respiratory surgical resection of the diseased pulmonary lobe or ligation of Society (ERS): Endorsed by: Association for European Paediatric and the anomalous artery is an acceptable choice. However, our Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37:67–119. patient had extensive lesions and unusual systemic arteries as [5] Jariwala P, Ramesh G, Sarat Chandra K. Congenital anomalous/aberrant feeding vessels of the AV fistulae, which precluded surgical systemic artery to pulmonary venous fistula: closure with vascular plugs treatment. During aortic angiography imaging for diagnosis, we & coil embolization. Indian Heart J 2014;66:95–103. used spring coils to embolize to the patient’s right internal [6] Singhi AK, Nicholson I, Francis E, et al. Anomalous systemic arterial mammary artery (the largest abnormal artery) for treatment supply to normal basal segment of the left lung. Heart Lung Circ 2011;20:357–61. (Fig. 4). However, no change in pulmonary pressure was detected

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Medicine – Wolters Kluwer Health

Published: Feb 1, 2018

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