Journal of Pediatric Cardiology and Cardiac Surgery 3(1): 35‒38 (2019)

Case Report

Eﬀectiveness of Lobectomy in a Pediatric Patient withPulmonary Hemorrhage and Hemoptysis Due toPulmonary Vein Restenosis after Congenital
Pulmonary Vein Stenosis Repair
Sadahiro Furui, MD1), Takaomi Minami, MD, PhD1), Mitsuru Seki, MD, PhD1),
Shun Suzuki, MD1), Kensuke Oka, MD1), Daisuke Matsubara, MD1),
Tomoyuki Sato, MD, PhD1), Koichi Kataoka, MD, PhD1), Shigeru Ono, MD, PhD2),
Masaaki Kawada, MD, PhD3), and Takanori Yamagata, MD, PhD1)
1) Department of Pediatrics, Jichi Childrenʼs Medical Center Tochigi, Tochigi, Japan

2) Department of Pediatric Surgery, Jichi Childrenʼs Medical Center Tochigi, Tochigi, Japan

3) Department of Pediatric Cardiovascular Surgery, Jichi Childrenʼs Medical Center Tochigi, Tochigi, Japan

Congenital pulmonary vein stenosis (CPVS) is extremely rare, accounting for 0.03% of cases of congenital heartdisease. The prognosis of CPVS is poor because the secondary pulmonary hypertension (PH) is progressive.
Treatments options include surgical repair using a sutureless technique and stent implantation; however, thestenosis often recurs. Herein, a boy with a partial anomalous pulmonary venous connection was treated forCPVS using a sutureless technique at the age of 1 year old; however, the stenosis recurred, and he experienced
repeated hemoptysis from the age of 2 years and 8 months. Because of the severity of his condition, surgery or acatheter intervention would have been challenging, and the bleeding points could not be identified. After detailed
investigation using cardiac catheterization and three-dimensional computed tomography, a partial resection of
the lower lobe of the right lung was performed, and hemoptysis was resolved. Compensatory enlargement of theremaining lobes maintained the patientʼs respiratory function with no mediastinal shift. There was no evidence ofhemoptysis during 5 years following the lobectomy, and he goes to school without exercise restrictions. Becauselung transplantation is not a viable treatment option in Japan, lobectomy may become a therapeutic option fora pulmonary hemorrhage caused by restenosis of the pulmonary vein after CPVS repair using a sutureless technique.

Keywords: congenital pulmonary vein stenosis, pulmonary hypertension, pulmonary hemorrhage, lung
lobectomy

Introduction
Congenital pulmonary vein stenosis (CPVS), firstreported by Reye et al. in 1951,1) is a congenital stenotic
disease of the ostia of the pulmonary veins in the left
atrium. This cardiac anomaly is extremely rare, accounting for 0.03% of cases of congenital heart disease.2) It has
been reported that 30‒80% of patients with CPVS haveother abnormalities such as a total or partial anomalous
pulmonary venous connection or cor triatriatum.2, 3)
The prognosis of CPVS is poor because the secondary
pulmonary hypertension (PH) is progressive. Since theeﬀectiveness of treatments for CPVS, such as surgicalrepair with a sutureless technique, balloon angioplasty,
stent implantation, and chemotherapy,4, 5) are limited,
the pulmonary vein restenosis occurs at a high rate.

Received: January 31, 2018; Accepted: November 13, 2018
Corresponding author: Sadahiro Furui, MD, Department of Pediatrics, Jichi Childrenʼs Medical center Tochigi, 3311‒1 Yakushiji,Shimotsuke, Tochigi 329‒0498, Japan
E-mail: r0945sf@jichi.ac.jp
doi: 10.24509/jpccs.190106© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery

36Fig. 1 a) CT at 8 months showing a partial anomalous pulmonary venous connection of the right upper pulmonaryvein. b, c) CT at 8 months showing stenosis of the ostium of the right inferior pulmonary vein into the left
atrium. d) Preoperative chest radiography. Diﬀuse congestion can be seen in both lungs, with severe congestion particularly evident in the right lung. e) Postoperative chest radiography showing the accumulationof pleural ﬂuid in the right lung. f) Chest radiography 2 years after surgery. Most of the pleural ﬂuid had beenabsorbed, and the upper and middle lobes of the lung had increased in size to compensate for the resectedportion.
Table 1 Results of cardiac catheterization
Pressure (mmHg)

mPA (s/d/m)
Lt. PA (s/d/m)
Rt. PA (s/d/m)
Lt. PAW
Rt. PAWLV
Ao (s/d/m)
Pp/Ps
Rp (Right)
Rp (Left)Rp (total)

At 9 months of age

At 1 year and 6 months of age

At 3 years and 5 months of age

47/19/28
47/19/28
47/19/28
101063/EDP 5
63/35/48
0.75

66/34/50
66/34/50
66/34/50
202067/EDP 8
67/37/51
1.0
52.6 Um2
9.91 Um2
8.34 Um2

42/17/29
42/14/27
42/17/29
U (15), L (18)U (26), L (25)70/EDP 11
70/44/55
0.60
38.9 Um2
3.60 Um2
3.33 Um2

The use of a pulmonary vasodilator alleviated the pulmonary vascular resistance and pulmonary hypertension.
mPA: Main PA

We report here a case of a 3-year-old boy with rightlower CPVS and a partial anomalous pulmonary venous
connection. The right lower CPVS was surgically
repaired using a sutureless technique at the age of 1 year
old, however, pulmonary vein stenosis (PVS) recurred,resulting in recurrent hemorrhages. We performed apartial resection of the lower lobe of the right lung totreat hemoptysis. Partial right lower lobectomy waseﬀective for the recurrent hemorrhage due to pulmonary
J Pediatr Cardiol Card Surg Vol. 3, No. 1 (2019)vein restenosis after CPVS repair.

CaseThe patient was a boy aged 3 years and 5 months.
He was born via a cesarean section at 39 weeksʼ gestation, with a birth weight of 2,354 g. He had no family
history of congenital heart disease. At 2 months of age,
he was diagnosed congestive heart failure with atrial
septal defect when he suﬀered from viral infection. At

37

6 months, he was referred to our institution for further
examination and treatment. Three-dimensional computed tomography (CT) revealed the right superior
partial anomalous pulmonary venous connection to thesuperior vena cava and the stenosis of the right inferior
pulmonary vein at the orifice (Fig. 1a‒c). No stenosis
was observed in the left superior pulmonary vein. At 9months, cardiac catheterization revealed that he had anelevated mean pulmonary arterial pressure (28 mmHg)
with a normal pulmonary artery wedge pressure bilaterally (10 mmHg). His left inferior pulmonary vein drained
into the left atrium via the left superior pulmonary vein.
The pulmonary to systemic flow ratio (Qp/Qs) was high,at 3.7, indicating that PH was due to increased pulmonary blood flow by atrial septal defect (Table 1).
At 1 year of age, he underwent surgical repair withsutureless technique for the right lower PVS and rerouting of the right upper pulmonary vein to the left atrium
using the modified Warden. The sinus venosus typearterial septal defect was 13 mm of diameter. The pulmonary vein branches were trimmed and cut back enoughto ensure suﬃcient large openings. The transected left
atrium wall and surrounding pericardium were thencircumferentially sutured to the pulmonary veins. Afterthe release of PVS, the pulmonary-to-systemic bloodpressure ratio (Pp/Ps) decreased from 0.75 to 0.35 withintraoperative measurement. In addition, a biopsy of
the right middle lung was performed. This revealed thethickening of the tunica media without hypoplasia of thepulmonary arteries or pulmonary vascular obstruction.
The smooth muscle cells in the pulmonary veins had
proliferated and formed the tunica media.
Echocardiography showed progressive right PV
restenosis from 3 months after surgery, therefore, weperformed cardiac catheterization when he was 1 year
and 6 months old. This examination revealed severe
pulmonary arterial hypertension and right PV stenosis.
Since there is no obstruction of left pulmonary vein,
we calculated right and left pulmonary vascular resistances separately using the results of lung perfusion
scintigraphy (right-to-left ratio was 15 : 85) to obtaineach pulmonary blood flow. Both pulmonary vascular
resistances were elevated with a large deference (right,52.6 units m2; left, 9.91 units m2). We judged that pulmonary arterial hypertension could be treated and initiatedbosentan, sildenafil, and home oxygen therapy after thecatheterization. Thereafter, pressure gradient of tricus-pid regurgitation by echocardiography decreased from
85 mmHg to 40 mmHg.
Despite the improvement of PH, he suﬀered fromfocal right lower lobe pneumonia complicated withpulmonary hemorrhage repeatedly from the age of 2years and 4 months. At 3 years and 5 months of age, we
performed further examinations including cardiac catheterization. On chest X-ray, there was diﬀuse pulmonary
congestion in both lung fields without pleural eﬀusion(Fig. 1d). The ejection fraction was 63%, and the ratioof right-to-left ventricular systolic blood pressure wasestimated to be 0.52 by echocardiography. The bloodflow velocity was 2.1 m/s in the right pulmonary veins
indicating the remaining of right PVS. On cardiac catheterization showed the descending aortic pressure and thepulmonary arterial pressure was 70/44 (55) mmHg and
42/17 (29) mmHg, respectively. The pulmonary vascular
resistances calculated using pulmonary blood flow ratio(right : left=3 : 97) by lung perfusion scintigraphy were
38.9 units m2 on the right and 3.60 units m2 on the left
(Table 1), indicating that the pulmonary vascular resistance was improved by medical intervention. Right pulmonary arteriography revealed diminished pulmonary
vascular bed, right lower PV obstruction (Movie).
These results showed the patientʼs ventilation andblood flow were primarily dependent on the left lung.
Bronchoscopy did not reveal the bleeding point in
the right main bronchus. The recurrent infection andhemoptysis in the lower lobe of the right lung wereconsidered life-threatening. Therefore, we decided toperform a partial resection of the lower lobe of the rightlung. The lobectomy proceeded without complication,and he was discharged 1 month after surgery. On X-ray,
unlike many adult cases right upper and middle lobes
increased in size to compensate for the resected portionof the right lower lobe without a mediastinal shift (Fig.
1e, f). The administration of bosentan, sildenafil werecontinued to treat the pulmonary arterial hypertension.
Although he was subsequently re-admitted twice forpneumonia, there was no evidence of hemoptysis for
5 years following the lobectomy. Oxygen saturation
is 96% on room air, and he continues oxygen therapy
only during night and goes to school without exercise
restrictions.

Discussion
This case yielded two main findings. First, a partial© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery

38

lobectomy can be eﬀective for treating hemoptysis andpulmonary hemorrhage due to pulmonary vein restenosis after a surgical repair of CPVS. Second, a partial
lobectomy in early childhood can preserve the respiratory reserve through compensatory development of theremaining lobes without causing a mediastinal shift.
CPVS shows various forms morphologically from a
focal stenosis of the pulmonary vein orifice to the wide
range of pulmonary vein hypoplasia. The symptoms ofCPVS and their severity depend on the degree of stenosis and its extent3, with common symptoms in early
infancy including poor weight gain, tachypnea, andrepeated bouts of respiratory infections. CPVS can also
cause hemoptysis in children,3) as was observed in thepresent case.
Most cases of CPVS are treated by surgical repair withsutureless technique or via cardiac catheterization, suchas stent implantation or balloon angioplasty.6) However,in cases of diﬀuse and progressive pulmonary vein stenosis, the eﬀect of these treatments is limited. Therefore,lung transplantation might be a useful treatment option
for CPVS. In a study by Holt et al.,2) 7 of 31 patients withbilateral CPVS underwent lung transplantation and 4 ofthose patients were received as their initial treatment.
Three of the 7 patients who underwent lung transplantation survived 2‒11 years after surgery. Since lung transplantation is not a common treatment option, partial
lobectomy can provide an eﬀective treatment option for
focal PVS especially in patients with severe congestion
and marked PH or hemoptysis.
When a partial lobectomy, common treatment foryoung patients with bronchiectasis or pulmonary
sequestration, is performed in early childhood, therespiratory reserve can be maintained because of thecompensatory development of the remaining lung lobes
without causing a mediastinal shift. Regarding CPVS
patients, one study reported the eﬀectiveness of pneumonectomy for treating patients with unilateral PVS,7)
however there have been few reports of a partial lobectomy being performed for the same purpose. Pneumonectomy markedly reduces the respiratory reserve function and may cause thoracic cavity collapse, resulting in
the mediastinum shift potentially causing the stenosis of
the main bronchus of the remaining lung through vertebral compression. These complications might become

J Pediatr Cardiol Card Surg Vol. 3, No. 1 (2019)

serious problems, especially after the removal of thetotal right lung. In the present case, only the right lower
lobe exhibited the destruction of the alveolar structuresand had no ventilatory capability because of severe PVS,
lung congestion, and repeated bouts of inflammation.
In addition, there was no pulmonary congestion in theright upper and middle lobes, and the pulmonary vein
drainage was maintained. For these reasons, we consider
the right lower lobe lobectomy is a better treatment
option rather than the right total pneumonectomy. The
lobectomy allowed compensatory development of theremaining lobes after surgery without causing a mediastinal shift, preserving the patientʼs respiratory reserve.

Conclusion
In a young patient with CPVS, a partial lobectomy
was eﬀective for treating a pulmonary hemorrhage dueto restenosis of the pulmonary vein after surgical repair
using a sutureless technique. This strategy improved thepatientʼs quality of life.
Conﬂicts of Interest
The authors declare that they have no conflict of interest.
NoteSupplementary movies are provided online for this article.

References
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phase II trial of vinblastine and methotrexate in multivessel intraluminal pulmonary vein stenosis in infants andchildren. Congenit Heart Dis 2011; 6: 608‒6235) Suzuki H, Niki T, Otagiri T, et al: Three cases of congenital
pulmonary vein stenosis. Pediatr Cardiol Cardiac Surg
2014; 30: 334‒3426) Kenji W: Catheter intervention for the treatment of postoperative pulmonary vein stenosis. Pediatr Cardiol Cardiac Surg 2014; 30: 350‒3527) Reid JM, Jamieson MP, Cowan MD: Unilateral pulmonaryvein stenosis. Br Heart J 1986; 55: 599‒601