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Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery
G Model
JCCASE-1161; No. of Pages 3 Journal of Cardiology Cases xxx (2019) xxx–xxx
Contents lists available at ScienceDirect
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case report
Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery Kota Kawada ((MD))a,b, Hirofumi Saiki ((MD))c,*, Manabu Kemmochi ((MD))a, Seiko Kuwata ((MD))c, Manabu Takanashi ((MD))c, Kagami Miyaji ((MD))d, Hideaki Senzaki ((MD))c a
Department of Neonatology, Kitasato University, Sagamiraha, Japan Kawada Pediatric Clinic, Shizuoka, Japan c Department of Pediatrics and Pediatric Cardiology, Kitasato University, Sagamihara, Japan d Department of Thoracic and Cardiovascular Surgery, Kitasato University, Sagamihara, Japan b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 July 2019 Received in revised form 28 November 2019 Accepted 15 December 2019
Isolated unilateral absence of the pulmonary artery (UAPA) is a congenital anomaly where involution of the extrapulmonary PA is insufficient and the intrapulmonary PA is only fed by the ductus arteriosus. Affected lung disorder causes complications years after ductus closure; thus, early diagnosis is of importance to avoid these complications. Here, we present the case of a male infant who was admitted to the neonatal intensive care unit because of transient tachypnea of the newborn and absence of the left PA (LPA) was indicated. Intensive echocardiography could detect neither the LPA nor the aortopulmonary collateral arteries to the left lung. Although the ductus was orthotopic with the right aortic arch, use of prostaglandin (PG) E1 unmasked the diagnosis of UAPA with bilateral ductus arteriosus. After ductal closure, delineation of the anatomy is not necessarily easy even with catheterization, whereas early use of PGE1 facilitates anatomical understanding by echocardiography, particularly early after birth. © 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Keywords: Absent pulmonary artery Bilateral ductus arteriosus Prostaglandin
Introduction Isolated unilateral absence of the pulmonary artery (UAPA), which is called an isolated PA of ductal origin, is a congenital lesion where the extrapulmonary PA is not properly developed, while the intrapulmonary PA is normal. Embryologically, inadequate involution of the proximal sixth arch and persistent communication of the distal sixth arch with the intrapulmonary artery cause discontinuation of blood supply to the unilateral PA as the ductus
* Corresponding author at: Department of Pediatrics and Pediatric Cardiology, Kitasato University, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan. E-mail address: [email protected] (H. Saiki).
closes. Although no clinical symptom may be observed for years, the variety of clinical presentation is known, including hemoptysis, recurrent respiratory infection, and pulmonary hypertension [1]. As such pulmonary disease develops silently but can be critical, early detection and optimal management to avoid it are of importance. Case report The patient was a boy who was uneventfully delivered vaginally at the gestational age of 38 weeks, with a weight of 2460 g. He was unexpectedly admitted to the neonatal intensive care unit because of transient tachypnea of the newborn, with a SpO2 of 90%, which easily increased to 100% with oxygen supplementation. Chest radiography revealed decreased vascularity in the left lung, while
https://doi.org/10.1016/j.jccase.2019.12.004 1878-5409/© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Kawada K, et al. Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery. J Cardiol Cases (2020), https://doi.org/10.1016/j.jccase.2019.12.004
G Model
JCCASE-1161; No. of Pages 3 K. Kawada et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
2
collateral artery feeding the left lung was identified. However, the tiny dimple in the brachiocephalic artery implied the possibility of a second ductus in the left lung, compelling us to initiate prostaglandin (PG) E1 infusion (Fig. 2B). Continuous cardiac murmur at the second interspace on the left sternal border became audible 8 h after PGE1 infusion, and subsequent echocardiography identified an arterial blood flow pattern from the brachiocephalic artery to the left lung (Fig. 3A,B). Repeated contrast-enhanced CT delineated the LPA branches from both the upper and lower lung; thus, the artery proximal to the LPA should be the second ductus, which led to a definite diagnosis of UAPA. A higher dose of PGE1 was unable to increase the LPA flow, so we planned a surgical reconstruction of the LPA. After trimming the ductus tissue, the LPA diameter was 3.5 mm, and an expanded polytetrafluoroethylene graft (5 mm in diameter with an 8-mm length) was interposed between the main PA and the intrapulmonary portion of the LPA. Although the postoperative course was uneventful, the graft flow became undetectable in a week, and percutaneous transluminal angioplasty was urgently performed. LPA flow was successfully recovered, and satisfactory vascular growth was observed (Fig. 3C).
Discussion
Fig. 1.
(A) Chest radiography image showing decreased vascularity in the left lung. (B) Right aortic arch with an orthotopic ductus. (C) Only the right pulmonary artery stemmed from the main pulmonary artery. aAo, ascending aorta; DA, ductus arteriosus; dAo, descending aorta; mPA, main pulmonary artery; RtPA, right pulmonary artery; SVC, supra vena cava.
the lung volume was preserved (Fig. 1A). Echocardiography could not identify the left PA (LPA); otherwise, no malformation was observed. Only the right PA (RPA) was stemmed from the main PA, which also communicated with the right-side aortic arch via a large ductus arteriosus with bidirectional blood flow (Fig. 1B and C). No artery was suspected to feed blood to the left lung, and left pulmonary venous return was unclear despite its identified vascular shape. Contrast-enhanced computed tomography (CT) revealed that the left lung and bronchus were normally structured and the main PA was orthotopic (Fig. 2A,B). Neither a LPA nor a
Fig. 2.
Isolated UAPA is rare congenital anomaly, with an incidence of 1 per 200,000 births. Its diagnosis in infancy is not easy because the patient is often asymptomatic, particularly after ductus closure. As most patients are supposed to develop symptoms of pulmonary hypertension or respiratory diseases [1], early therapeutic intervention to rehabilitate the PA is vitally important to secure lung perfusion and volume [2,3]. Part of the UAPA is complicated by bilateral ductus arteriosus [4,5]. In the present case, screening echocardiography incidentally detected the absence of the LPA, which led us to attempt finding the LPA. Although the right-sided ductus made the diagnosis confusing, structurally normal left pulmonary veins and the dimple in the brachiocephalic artery were key to suspect the possibility of a second ductus, that is, bilateral ductus arteriosus (Fig. 2c). McElhinney et al. reported a similar experience, but the ductus was patent in 8 months [4]. Of 18 cases, only 12 had a delineated anatomy by cardiac catheterization with pulmonary wedge angiography if the ductus was absent [6]. To our knowledge, this is the first reported case of reopening of a closed ductus in neonatal UAPA with bilateral ductus arteriosus.
(A) Contrast CT image cannot indicate Lt PA raising from mPA. The circle indicates the missing left pulmonary artery. (B) Reconstructed 3-D image showing a dimple in the brachiocephalic artery. The right aortic arch with an orthotopic ductus is also shown. (C) Schema of the vessel relation (deformation based on schema of pharyngeal arch) is shown. aAo, ascending aorta; BCA, brachiocephalic artery; CA, carotid artery; CT, computed tomography; DA, ductus arteriosus; dAo, descending aorta; Lt, left; m, main; Rt, right; PV, pulmonary vein.
Please cite this article in press as: Kawada K, et al. Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery. J Cardiol Cases (2020), https://doi.org/10.1016/j.jccase.2019.12.004
G Model
JCCASE-1161; No. of Pages 3 K. Kawada et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
Fig. 3.
3
(A,B) After PGE1 administration, blood flow to the left lung is detected. (C) Pulmonary angiogram shows favorable growth of the left pulmonary artery after successful graft-interpose and percutaneous angioplasty. Brachiocephalic a., brachiocephalic artery; Lt, left; m, main; PA, pulmonary artery; PGE1, prostaglandin E1; PV, pulmonary vein; Rt, right.
Neonatal surgery was planned because an additional dose of PGE1 was ineffective to further increase blood flow to the LPA. Although unifocalization subsequent to systemic-pulmonary shunt is often recommended for UAPA, primary unifocalization was selected in this case, assuming that the LPA had been fed adequately until birth. This strategy is further supported by the fact that a systemic-pulmonary shunt can be a risk factor for later pulmonary hypertension [7] and by the report that intervention in younger age is a determinant of neither increasing complications nor subsequent interventions [2,6]. Indeed, as compared with primary repair, patients with staged repair had significantly low pulmonary perfusion in the affected lung and underwent more surgical and total interventions despite older age (2.4 months vs 35 months). Importantly, only 75% (6 out of 8) of patients could undergo subsequent unifocalization [6]. Taken together, early diagnosis was most important, by which early unifocalization can be attained to secure an ideal growth of the PA [2,6] and lungs [3]. Batlivala et al. reported that 89% of their patients were diagnosed on the basis of lung- or heart-related symptoms, including respiratory distress or heart murmur [6]. As was seen in our patient, a single chest radiography examination clearly demonstrated a decreased vasculature. Accordingly, even with mild symptoms, simply having a differential diagnosis of UAPA would greatly help make a correct diagnosis. If UAPA is suspected, early initiation of PGE1 administration, particularly in
neonates, might be useful for unraveling the anatomy by reopening the ductus, even with bilateral ductus arteriosus. Conflict of Interest None. References [1] Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002;122:1471–7. [2] Kim GB, Ban JE, Bae EJ, Noh CI, Kim WH, Lee JR, et al. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg 2011;141:171–8. [3] Alison M, Garel L, Bigras JL, Dery J, Lapierre C. Unilateral absence of pulmonary artery in children: bronchovascular anatomy, natural course and effect of treatment on lung growth. Pediatr Radiol 2011;41:459–68. [4] McElhinney DB, Hoydu AK, Chin AJ, Weinberg PM. Right-sided aortic arch with bilateral ductus: a rare case of nonconfluent pulmonary arteries without associated cardiac anomalies. J Thorac Cardiovasc Surg 2000;119:849–51. [5] Freedom RM, Moes CA, Pelech A, Smallhorn J, Rabinovitch M, Olley PM, et al. Bilateral ductus arteriosus (or remnant): an analysis of 27 patients. Am J Cardiol 1984;53:884–91. [6] Batlivala SP, McElhinney DB, Pigula FA, Marshall AC. Isolated pulmonary artery arising from a duct: a single-center review of diagnostic and therapeutic strategies. J Thorac Cardiovasc Surg 2014;148:2245–52. [7] Agrawal H, Petit CJ, Miro J, Miranda CD, Kenny D, Justino H. Contralateral pulmonary hypertension following resuscitation of unilateral ductal origin of a pulmonary artery: a multi-institutional review. Pediatr Cardiol 2018;39:71–8.
Please cite this article in press as: Kawada K, et al. Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery. J Cardiol Cases (2020), https://doi.org/10.1016/j.jccase.2019.12.004
JCCASE-1161; No. of Pages 3 Journal of Cardiology Cases xxx (2019) xxx–xxx
Contents lists available at ScienceDirect
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case report
Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery Kota Kawada ((MD))a,b, Hirofumi Saiki ((MD))c,*, Manabu Kemmochi ((MD))a, Seiko Kuwata ((MD))c, Manabu Takanashi ((MD))c, Kagami Miyaji ((MD))d, Hideaki Senzaki ((MD))c a
Department of Neonatology, Kitasato University, Sagamiraha, Japan Kawada Pediatric Clinic, Shizuoka, Japan c Department of Pediatrics and Pediatric Cardiology, Kitasato University, Sagamihara, Japan d Department of Thoracic and Cardiovascular Surgery, Kitasato University, Sagamihara, Japan b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 July 2019 Received in revised form 28 November 2019 Accepted 15 December 2019
Isolated unilateral absence of the pulmonary artery (UAPA) is a congenital anomaly where involution of the extrapulmonary PA is insufficient and the intrapulmonary PA is only fed by the ductus arteriosus. Affected lung disorder causes complications years after ductus closure; thus, early diagnosis is of importance to avoid these complications. Here, we present the case of a male infant who was admitted to the neonatal intensive care unit because of transient tachypnea of the newborn and absence of the left PA (LPA) was indicated. Intensive echocardiography could detect neither the LPA nor the aortopulmonary collateral arteries to the left lung. Although the ductus was orthotopic with the right aortic arch, use of prostaglandin (PG) E1 unmasked the diagnosis of UAPA with bilateral ductus arteriosus. After ductal closure, delineation of the anatomy is not necessarily easy even with catheterization, whereas early use of PGE1 facilitates anatomical understanding by echocardiography, particularly early after birth.
Keywords: Absent pulmonary artery Bilateral ductus arteriosus Prostaglandin
Introduction Isolated unilateral absence of the pulmonary artery (UAPA), which is called an isolated PA of ductal origin, is a congenital lesion where the extrapulmonary PA is not properly developed, while the intrapulmonary PA is normal. Embryologically, inadequate involution of the proximal sixth arch and persistent communication of the distal sixth arch with the intrapulmonary artery cause discontinuation of blood supply to the unilateral PA as the ductus
* Corresponding author at: Department of Pediatrics and Pediatric Cardiology, Kitasato University, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan. E-mail address: [email protected] (H. Saiki).
closes. Although no clinical symptom may be observed for years, the variety of clinical presentation is known, including hemoptysis, recurrent respiratory infection, and pulmonary hypertension [1]. As such pulmonary disease develops silently but can be critical, early detection and optimal management to avoid it are of importance. Case report The patient was a boy who was uneventfully delivered vaginally at the gestational age of 38 weeks, with a weight of 2460 g. He was unexpectedly admitted to the neonatal intensive care unit because of transient tachypnea of the newborn, with a SpO2 of 90%, which easily increased to 100% with oxygen supplementation. Chest radiography revealed decreased vascularity in the left lung, while
https://doi.org/10.1016/j.jccase.2019.12.004 1878-5409/© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Kawada K, et al. Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery. J Cardiol Cases (2020), https://doi.org/10.1016/j.jccase.2019.12.004
G Model
JCCASE-1161; No. of Pages 3 K. Kawada et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
2
collateral artery feeding the left lung was identified. However, the tiny dimple in the brachiocephalic artery implied the possibility of a second ductus in the left lung, compelling us to initiate prostaglandin (PG) E1 infusion (Fig. 2B). Continuous cardiac murmur at the second interspace on the left sternal border became audible 8 h after PGE1 infusion, and subsequent echocardiography identified an arterial blood flow pattern from the brachiocephalic artery to the left lung (Fig. 3A,B). Repeated contrast-enhanced CT delineated the LPA branches from both the upper and lower lung; thus, the artery proximal to the LPA should be the second ductus, which led to a definite diagnosis of UAPA. A higher dose of PGE1 was unable to increase the LPA flow, so we planned a surgical reconstruction of the LPA. After trimming the ductus tissue, the LPA diameter was 3.5 mm, and an expanded polytetrafluoroethylene graft (5 mm in diameter with an 8-mm length) was interposed between the main PA and the intrapulmonary portion of the LPA. Although the postoperative course was uneventful, the graft flow became undetectable in a week, and percutaneous transluminal angioplasty was urgently performed. LPA flow was successfully recovered, and satisfactory vascular growth was observed (Fig. 3C).
Discussion
Fig. 1.
(A) Chest radiography image showing decreased vascularity in the left lung. (B) Right aortic arch with an orthotopic ductus. (C) Only the right pulmonary artery stemmed from the main pulmonary artery. aAo, ascending aorta; DA, ductus arteriosus; dAo, descending aorta; mPA, main pulmonary artery; RtPA, right pulmonary artery; SVC, supra vena cava.
the lung volume was preserved (Fig. 1A). Echocardiography could not identify the left PA (LPA); otherwise, no malformation was observed. Only the right PA (RPA) was stemmed from the main PA, which also communicated with the right-side aortic arch via a large ductus arteriosus with bidirectional blood flow (Fig. 1B and C). No artery was suspected to feed blood to the left lung, and left pulmonary venous return was unclear despite its identified vascular shape. Contrast-enhanced computed tomography (CT) revealed that the left lung and bronchus were normally structured and the main PA was orthotopic (Fig. 2A,B). Neither a LPA nor a
Fig. 2.
Isolated UAPA is rare congenital anomaly, with an incidence of 1 per 200,000 births. Its diagnosis in infancy is not easy because the patient is often asymptomatic, particularly after ductus closure. As most patients are supposed to develop symptoms of pulmonary hypertension or respiratory diseases [1], early therapeutic intervention to rehabilitate the PA is vitally important to secure lung perfusion and volume [2,3]. Part of the UAPA is complicated by bilateral ductus arteriosus [4,5]. In the present case, screening echocardiography incidentally detected the absence of the LPA, which led us to attempt finding the LPA. Although the right-sided ductus made the diagnosis confusing, structurally normal left pulmonary veins and the dimple in the brachiocephalic artery were key to suspect the possibility of a second ductus, that is, bilateral ductus arteriosus (Fig. 2c). McElhinney et al. reported a similar experience, but the ductus was patent in 8 months [4]. Of 18 cases, only 12 had a delineated anatomy by cardiac catheterization with pulmonary wedge angiography if the ductus was absent [6]. To our knowledge, this is the first reported case of reopening of a closed ductus in neonatal UAPA with bilateral ductus arteriosus.
(A) Contrast CT image cannot indicate Lt PA raising from mPA. The circle indicates the missing left pulmonary artery. (B) Reconstructed 3-D image showing a dimple in the brachiocephalic artery. The right aortic arch with an orthotopic ductus is also shown. (C) Schema of the vessel relation (deformation based on schema of pharyngeal arch) is shown. aAo, ascending aorta; BCA, brachiocephalic artery; CA, carotid artery; CT, computed tomography; DA, ductus arteriosus; dAo, descending aorta; Lt, left; m, main; Rt, right; PV, pulmonary vein.
Please cite this article in press as: Kawada K, et al. Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery. J Cardiol Cases (2020), https://doi.org/10.1016/j.jccase.2019.12.004
G Model
JCCASE-1161; No. of Pages 3 K. Kawada et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
Fig. 3.
3
(A,B) After PGE1 administration, blood flow to the left lung is detected. (C) Pulmonary angiogram shows favorable growth of the left pulmonary artery after successful graft-interpose and percutaneous angioplasty. Brachiocephalic a., brachiocephalic artery; Lt, left; m, main; PA, pulmonary artery; PGE1, prostaglandin E1; PV, pulmonary vein; Rt, right.
Neonatal surgery was planned because an additional dose of PGE1 was ineffective to further increase blood flow to the LPA. Although unifocalization subsequent to systemic-pulmonary shunt is often recommended for UAPA, primary unifocalization was selected in this case, assuming that the LPA had been fed adequately until birth. This strategy is further supported by the fact that a systemic-pulmonary shunt can be a risk factor for later pulmonary hypertension [7] and by the report that intervention in younger age is a determinant of neither increasing complications nor subsequent interventions [2,6]. Indeed, as compared with primary repair, patients with staged repair had significantly low pulmonary perfusion in the affected lung and underwent more surgical and total interventions despite older age (2.4 months vs 35 months). Importantly, only 75% (6 out of 8) of patients could undergo subsequent unifocalization [6]. Taken together, early diagnosis was most important, by which early unifocalization can be attained to secure an ideal growth of the PA [2,6] and lungs [3]. Batlivala et al. reported that 89% of their patients were diagnosed on the basis of lung- or heart-related symptoms, including respiratory distress or heart murmur [6]. As was seen in our patient, a single chest radiography examination clearly demonstrated a decreased vasculature. Accordingly, even with mild symptoms, simply having a differential diagnosis of UAPA would greatly help make a correct diagnosis. If UAPA is suspected, early initiation of PGE1 administration, particularly in
neonates, might be useful for unraveling the anatomy by reopening the ductus, even with bilateral ductus arteriosus. Conflict of Interest None. References [1] Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002;122:1471–7. [2] Kim GB, Ban JE, Bae EJ, Noh CI, Kim WH, Lee JR, et al. Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery. J Thorac Cardiovasc Surg 2011;141:171–8. [3] Alison M, Garel L, Bigras JL, Dery J, Lapierre C. Unilateral absence of pulmonary artery in children: bronchovascular anatomy, natural course and effect of treatment on lung growth. Pediatr Radiol 2011;41:459–68. [4] McElhinney DB, Hoydu AK, Chin AJ, Weinberg PM. Right-sided aortic arch with bilateral ductus: a rare case of nonconfluent pulmonary arteries without associated cardiac anomalies. J Thorac Cardiovasc Surg 2000;119:849–51. [5] Freedom RM, Moes CA, Pelech A, Smallhorn J, Rabinovitch M, Olley PM, et al. Bilateral ductus arteriosus (or remnant): an analysis of 27 patients. Am J Cardiol 1984;53:884–91. [6] Batlivala SP, McElhinney DB, Pigula FA, Marshall AC. Isolated pulmonary artery arising from a duct: a single-center review of diagnostic and therapeutic strategies. J Thorac Cardiovasc Surg 2014;148:2245–52. [7] Agrawal H, Petit CJ, Miro J, Miranda CD, Kenny D, Justino H. Contralateral pulmonary hypertension following resuscitation of unilateral ductal origin of a pulmonary artery: a multi-institutional review. Pediatr Cardiol 2018;39:71–8.
Please cite this article in press as: Kawada K, et al. Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery. J Cardiol Cases (2020), https://doi.org/10.1016/j.jccase.2019.12.004