Year : 2020  |  Volume : 13  |  Issue : 3  |  Page : 274--276

“Hurdles at each step:” Aberrant lobar pulmonary artery in an infant with severe aortic stenosis and coarctation – A unique triad


CR Pruthvi, V Krishna Santosh, Krishna Prasad Nevali, Dinakar Bootla, Sanjeev Naganur 
 Department of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Sanjeev Naganur
Department of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh
India




How to cite this article:
Pruthvi C R, Santosh V K, Nevali KP, Bootla D, Naganur S. “Hurdles at each step:” Aberrant lobar pulmonary artery in an infant with severe aortic stenosis and coarctation – A unique triad.Ann Pediatr Card 2020;13:274-276


How to cite this URL:
Pruthvi C R, Santosh V K, Nevali KP, Bootla D, Naganur S. “Hurdles at each step:” Aberrant lobar pulmonary artery in an infant with severe aortic stenosis and coarctation – A unique triad. Ann Pediatr Card [serial online] 2020 [cited 2020 Sep 20 ];13:274-276
Available from: http://www.annalspc.com/text.asp?2020/13/3/274/286465


Full Text



Sir,

Systemic arterialization of the lung without pulmonary sequestration is the rarest form of anomalous systemic arterial supply to the lung. Associated anomalies are very uncommon. Hereby, we describe a unique triad of aberrant systemic artery to lower lobe of the right lung, bicuspid aortic valve with severe valvular aortic stenosis, and coarctation of the aorta. This association has never been described in the past. We discuss the management options in this situation.

A 7-month-old boy presented to us with feeding difficulties and forehead sweating with no previous hospitalizations. He had apparently normal growth and development. All four limbs showed a saturation of 95%. There was mild tachypnea with recessions. There was a brachial-femoral pulse delay with blood pressure difference of 12–14 mmHg between the upper and the lower limbs. There was no cardiomegaly [Figure 1], with normal second heart sound and no additional sounds. There was an ejection systolic murmur of grade 4/6 at the aortic area.{Figure 1}

Electrocardiogram [Figure 2] showed normal sinus rhythm, normal axis and intervals for age and no ventricular hypertrophy. Echocardiography [Figure 3] showed bicuspid aortic valve, severe valvular aortic stenosis (gradient 74/42 mmHg), no aortic regurgitation, moderate postsubclavian coarctation (gradient 14 mmHg) with a vessel arising from descending thoracic aorta (DTA) and going to the right lung, good biventricular function. Cardiac computed tomography (CT) [Figure 4] showed discrete severe coarctation of the aorta, an aberrant vessel was found arising from DTA at T8 level, supplying the right posterior basal segments. There was no evidence of sequestration of the lung.{Figure 2}{Figure 3}{Figure 4}

Hence, he was planned for balloon aortic valvotomy (BAV) and assess the coarctation after aortic stenosis has been relieved along with the evaluation of aberrant artery from DTA supplying the right lung further.

It was easy to cross the coarctation segment with Judkins right diagnostic catheter (5Fr).

The aortic root hand injection [Figure 5] showed bicuspid aortic valve, no aortic regurgitation, normal origin of coronaries, and 14-mm aortic annulus with coarctation of the aorta. Hence, the successful BAV was performed using 12-mm TYSHAK II balloon with single dilatation [Figure 5]. In post-BAV, there was no significant residual aortic stenosis and no aortic regurgitation. Gradient across coarctation increased to 18 mmHg with diastolic spillage on echocardiogram [Figure 6]. The aberrant vessel was demonstrated to arise from DTA at the level of T8 and supplying right posterior basal segments [Figure 5]. The levophase showed a normal drainage of the pulmonary veins. The right ventricle angiogram showed probable dual supply to the segments [Figure 5].{Figure 5}{Figure 6}

Systemic arterialization of the lung without pulmonary sequestration is the rarest form of anomalous systemic arterial supply to the lung.[1],[2] This condition is characterized by an aberrant systemic arterial branch which can arise from the thoracic portion of the descending aorta or less commonly from the abdominal aorta or coeliac axis, supplying an area of lung parenchyma with normal bronchopulmonary system.[2],[3] The left lower lobe is most often involved, and there is commonly an atresia of the corresponding pulmonary artery with a normal venous drainage.[1] Associated anomalies are uncommon in systemic arterialization of the normal lung. Other associated malformations are seen in 14%–59% of cases, such as diaphragmatic hernia, congenital cystic adenomatoid malformation, and congenital heart disease.[1],[4]

Interestingly, our case presents an anomalous systemic arterial supply to a nonsequestered portion of the right lower lobe in association with normal right pulmonary artery with dual blood supply to the right lower lobe from the right pulmonary artery and aberrant systemic artery associated with congenital heart disease (bicuspid aortic valve with severe aortic stenosis and coarctation of the aorta). We could not find any similar association after extensive literature review.

Persistent remnant of the embryonic connection between the aorta and the pulmonary parenchyma is thought to be responsible. The prevalence of anomalous arterial supply to the lung without sequestration is very low with only ten cases reported in all English and Chinese literature up to 1994.[5] The average age incidence was 21.2 years. Male gender with a ratio of 2:1, left side and DTA as the aberrant arterial origin were predominant. Systemic arterial supply to the right lower lobe comprised 16% of cases.[3]

Most of the patients are asymptomatic; however, recurrent pulmonary infection, heart failure due to left-to-left shunt and hemoptysis are possible clinical presentations.[3] CT angiography can clearly show the origin of the aberrant systemic artery. MRI has also been reported to be useful.[5]

Treatment is recommended in both symptomatic and asymptomatic patients to prevent hemoptysis. Various treatment modalities have been described, including lobectomy or segmentectomy, systemic artery to pulmonary artery anastomosis, and endovascular treatment. It is very important to differentiate this condition from classic sequestration, as it may allow nonsurgical management (angiographic embolization) of the aberrant systemic artery.[4]

Our infant successfully underwent balloon dilatation of aortic valve for severe aortic stenosis. However, the unexpected finding of this anomaly merits further intervention (angiographic embolization) for the same at a later date to prevent complications. This may well be thefirst case report with a unique triad of systemic artery to the right lower lung, bicuspid aortic valve with severe aortic stenosis, and coarctation of the aorta.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorshipw

Nil.

Conflict of interest

There are no conflict of interest.

References

1Brühlmann W, Weishaupt D, Goebel N, Imhof E. Therapeutic embolization of a systemic arterialization of lung without sequestration. Eur Radiol 1998;8:355-8.
2Flisak ME, Chandrasekar AJ, Marsan RE, Ali MM. Systemic arterialization of lung without sequestration. AJR Am J Roentgenol 1982;138:751-3.
3Yamanaka A, Hirai T, Fujimoto T, Hase M, Noguchi M, Konishi F. Anomalous systemic arterial supply to normal basal segments of the left lower lobe. Ann Thorac Surg 1999;68:332-8.
4Agarwal PP, Matzinger FR, Seely JM, Rasuli P, Shamji FM. An unusual case of systemic arterial supply to the lung with bronchial atresia. AJR Am J Roentgenol 2005;185:150-3.
5Muñoz JJ, García JA, Bentabol M, Padín MI, Serrano F. Endovascular treatment of hemoptysis by abnormal systemic pulmonary artery supply. Cardiovasc Intervent Radiol 2008;31:427-30.