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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 4  |  Page : 324-327

A case of subvalvular aortic stenosis with pancytopenia: A nightmare for cardiac surgeons


Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, Jaipur, Rajasthan, India

Date of Submission31-May-2020
Date of Acceptance20-Aug-2020
Date of Web Publication24-Dec-2020

Correspondence Address:
Dr. Sharma Dhruva
Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJAM.IJAM_67_20

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  Abstract 


An 18-year-old boy presented with difficulty in breathing since birth. He had ejection systolic murmur heard loudest in the right second intercostal space radiating to the right carotids. On blood examination, he had severe pancytopenia. Electrocardiogram revealed left ventricular hypertrophy with a prominent Q-wave. On ultrasonography, it was revealed that the patient had splenomegaly with multiple collaterals. Echocardiography showed a tricuspid aortic valve with three cusps with a subaortic membrane. Concomitant splenectomy and subaortic membrane excision on cardiopulmonary bypass under general anesthesia was done. His platelet counts recovered soon after splenectomy.
The following core competencies are addressed in this article: Patient care and procedural skills, Medical knowledge, and Systems-based practice.

Keywords: Extrahepatic portal venous hypertension, pancytopenia, subvalvular aortic stenosis


How to cite this article:
Dhruva S, Sunil D, Anil S, Saurabh M. A case of subvalvular aortic stenosis with pancytopenia: A nightmare for cardiac surgeons. Int J Acad Med 2020;6:324-7

How to cite this URL:
Dhruva S, Sunil D, Anil S, Saurabh M. A case of subvalvular aortic stenosis with pancytopenia: A nightmare for cardiac surgeons. Int J Acad Med [serial online] 2020 [cited 2021 Jan 24];6:324-7. Available from: https://www.ijam-web.org/text.asp?2020/6/4/324/304602




  Introduction Top


Subvalvular aortic stenosis (AS), though a rare disorder seen in infants, is still considered an acquired condition.[1] It is correlated with other cardiac malformations in 50%–65% of cases such as ventricular septal defect (20%), patent ductus arteriosus (34%), pulmonic stenosis (9%), aortic coarctation (23%), and other miscellaneous lesions (14%).[2] Patients with pancytopenia are more prone to bleeding and infection during surgery.[3] Splenomegaly-induced thrombocytopenia has been chronicled previously also, but its association with subvalvular AS and pancytopenia is very rare.

Here, we report a rare case of severe AS with pancytopenia due to extrahepatic portal venous obstruction. A multitude of AS cases have been reported till date, but this is an unusual case as it is a nightmare to plan an open-heart surgery with reduced platelet counts.


  Case Report Top


An 18-year-old boy presented with difficulty in breathing since birth to the Cardiovascular and Thoracic Surgery Outpatient Department at SMS Hospital, Jaipur. On local examination, his heart rate was 76 beats/min, blood pressure was 90/60 mmHg in the right upper arm in lying down position, and respiratory rate was 16/minute. He had ejection systolic murmur heard loudest in the right second intercostal space radiating to the right carotids. On blood examination, he had severe pancytopenia; hemoglobin level was 7.2 g/dL, total leukocyte count was 1.96/mm3, platelet count was 0.26 lakhs/mL, and packed cell volume was 24.8%. Investigations were done at an advanced hematology laboratory, which showed adequate marrow function. Cardiomegaly was manifested on a chest X-ray with cardiothoracic ratio (CT ratio) >5 [Figure 1].
Figure 1: X-ray film depicting cardiomegaly with cardiothoracic ratio (CT ratio) >5

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Electrocardiogram revealed left ventricular hypertrophy with a prominent Q-wave. On ultrasonography, it was revealed that the patient had splenomegaly (length of the spleen was 19 cm) with multiple collaterals. On echocardiography, an aortic valve with three cusps with a subaortic membrane was seen while aortic regurgitation was absent. The mean pressure gradient was 64 mmHg and Vmax was 5.6 m/s. Left ventricular ejection fraction of the patient was 60%. A thorough preoperative assessment and intensive preparation of the patient was ensured.

Written informed consent was sought from the patient before surgery. When consultation from the internal medicine department was sought, the patient was investigated further. The investigation revealed extrahepatic portal venous obstruction, which was due to splenic consumption of white blood cells, red blood cells (RBCs), and platelets, and he had pancytopenia. The patient was advised concomitant splenectomy before the open-heart surgery for subvalvular AS. Consultation with a general surgeon was made, and surgery was planned accordingly so that splenectomy followed by open-heart surgery could be done. Prior to surgery, adequate arrangement for packed RBCs, single-donor platelets, random donor platelets, and whole blood was ensured. Intraoperative photograph showed splenectomy, as depicted in [Figure 2].
Figure 2: Intraoperative picture showing splenomegaly (length of the spleen is 19 cm)

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Systemic heparinization was done after sternotomy. The patient underwent cardiopulmonary bypass after central cannulation. Antegrade del-Nido cardioplegia solution was used with moderate hypothermia. The aorta was cross-clamped and aortotomy was done. When explored intraoperatively, the aortic valve was normal, but there was a subaortic membrane present, as shown in [Figure 3]. The aortic valve was examined and the subaortic membrane was identified and excised. No septal hypertrophy was seen. The patency of the aortic valve was checked. On table, the platelet count was 50,000/μL and immediately after splenectomy, a blood sample was sent for platelet count which showed an increase in the platelet count to 140,000/μL. Concomitant splenectomy followed by subaortic membrane excision under cardiopulmonary bypass under general anesthesia was done. Ceftriaxone was administered as a prophylaxis to prevent sepsis and opportunistic postsplenectomy infections. Immediately, the patient was shifted to the intensive care unit for further management. Two mediastinal drains and one abdominal drain were kept in situ. Hemodynamic monitoring with a close watch for drain output was done. Safe extubation was performed within 10 h. The postoperative period was uneventful. Pneumococcal, meningococcal, and Hemophilus influenza immunization were given as postprocedural immunization. The patient in the postoperative period is doing well with no limitation of activities, the counts have become normal, and two-dimensional echocardiography shows well-preserved ejection fraction.
Figure 3: Transverse aortotomy with a subaortic membrane. LCC = Left coronary cusp

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  Discussion Top


In most cases of subvalvular AS, also called subaortic stenosis, a muscular membrane is usually present just below the aortic valve, which causes a fixed obstruction to the blood flow across the left ventricular outflow tract.[1]

Subvalvular AS is the second most common type of AS which is responsible for approximately 1% of all congenital heart defects (8 in 10,000 births) and 15%–20% of all fixed left ventricular outflow tract obstructive lesions.

A male preponderance is seen in subvalvular AS with a male-to-female ratio of 2:1, and it is prevalent in 6.5% of all adult congenital heart disease patients.[4],[5],[6]

Exertional dyspnea is the most common symptom seen in 40% of symptomatic patients, while other commonly observed symptoms are angina, effort syncope, presyncope, orthopnea, and sudden cardiac death.

The test of choice to diagnose subvalvular AS is echocardiography, and surgical correction is the gold standard treatment modality.[7]

Kawai et al. reported a case of aplastic anemia involving pancytopenia caused by bone marrow insufficiency. They concluded that patients who are at high risk for surgical aortic valve replacement can be candidates for transcatheter aortic valve implantation.[3] Gangwani et al. documented a case of perioperative management of myelodysplastic syndrome for cardiac surgery requiring cardiopulmonary bypass.[8]

It was a demanding case as it was a challenge for the cardiac surgeon and the whole team to plan an open-heart surgery in such a case with pancytopenia. Moreover, it is investigated that, in open-heart surgery due to the use of heparin and other factors patient might bleed to death.

The association of subaortic stenosis and portal hypertension-related pancytopenia is in fact incidental and not syndromic. However, surgical approach is the optimal strategy considering the risk when compared to a less invasive interventional approach.

Moreover, in that case, the calculated risk for staged procedure was found to be more in view of severe subvalvular AS. Hence, after team discussion involving a cardiovascular and thoracic surgeon, a cardiac anesthetist, and a general surgeon, it was decided to do it as a single procedure.

Nevertheless, congenital subvalvular stenosis can be safely and efficiently managed using balloon dilation of the subvalvular membrane.[9]


  Conclusions Top


We suggest a multidisciplinary team approach including a cardiac surgeon, a general surgeon, a physician, a pediatrician, a cardiologist, a pathologist, a radiologist, and a neonatologist for the treatment of subvalvular AS. Calculated risk assessment with a good teamwork involving a well-coordinated, well-organized, and integrated approach can lead to a platonic landscape management of difficult clinical situations.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent documents. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published, and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Ethical conduct of research

The authors declare that this scientific work complies with reporting quality, formatting, and reproducibility guidelines set forth by the EQUATOR Network. The authors also attest that this clinical investigation did not require institutional review board/ethics committee review. For this work, formal consent of the patient was obtained.



 
  References Top

1.
Mulla S, Siddiqui WJ. Subaortic Stenosis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; January, 2019. Available from: https://www.ncbi.nlm.nih. gov/books/NBK526085/. [Last updated on 2019 Jul 04].  Back to cited text no. 1
    
2.
Choi JY, Sullivan ID. Fixed subaortic stenosis: Anatomical spectrum and nature of progression. Br Heart J 1991;65:280-6.  Back to cited text no. 2
    
3.
Kawai Y, Toyoda Y, Kimura H, Horigome M, Tsuda Y, Takemura T. Transcatheter aortic valve implantation in a patient with aplastic anemia. J Cardiol Cases 2017;16:213-5.  Back to cited text no. 3
    
4.
Kitchiner D, Jackson M, Malaiya N, Walsh K, Peart I, Arnold R. Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): A study of 313 patients. Br Heart J 1994;71:588-95.  Back to cited text no. 4
    
5.
Liu CW, Hwang B, Lee BC, Lu JH, Meng LC. Aortic stenosis in children: 19-year experience. Zhonghua Yi Xue Za Zhi (Taipei) 1997;59:107-13.  Back to cited text no. 5
    
6.
Oliver JM, González A, Gallego P, Sánchez-Recalde A, Benito F, Mesa JM. Discrete subaortic stenosis in adults: Increased prevalence and slow rate of progression of the obstruction and aortic regurgitation. J Am Coll Cardiol 2001;38:835-42.  Back to cited text no. 6
    
7.
Devabhaktuni SR, Chakfeh E, Malik AO, Pengson JA, Rana J, Ahsan CH. Subvalvular aortic stenosis: A review of current literature. Clin Cardiol 2018;41:131-6.  Back to cited text no. 7
    
8.
Gangwani AL, Hamid M, Naz F. Aortic valve replacement in a patient with pancytopenia secondary to myelodysplastic syndrome. J Pak Med Assoc 2014;64:207-9.  Back to cited text no. 8
    
9.
de Lezo JS, Romero M, Segura J, Pan M, de Lezo JS, Pavlovic D, et al. Long-term outcome of patients with isolated thin discrete subaortic stenosis treated by balloon dilation: A 25-year study. Circulation 2011;124:1461-8.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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