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| CASE REPORT |
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| Year : 2023 | Volume
: 28
| Issue : 2 | Page : 188-192 |
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Unruptured sinus of valsalva aneurysm coexisting with ventricular septal defect
Nneka C Udora1, Amamihechineke G Ojiakor1, Nelson I Oguanobi2, Raphael C Anakwue2
1 Department of Medicine, University of Nigeria Teaching Hospital, Ituku/Ozalla, Enugu, Nigeria
2 Department of Pharmacology and Therapeutics, Faculty of Medical Sciences, University of Nigeria, Enugu Campus, Enugu, Nigeria
| Date of Submission | 19-Sep-2022 |
| Date of Decision | 30-Nov-2022 |
| Date of Acceptance | 05-Feb-2023 |
| Date of Web Publication | 21-Mar-2023 |
Correspondence Address:
Nneka C Udora
Department of Medicine, University of Nigeria Teaching Hospital, Ituku/Ozalla, Enugu, Enugu State
Nigeria
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ijmh.IJMH_61_22
Unruptured sinus of valsalva aneursym is an incidental, relatively rare finding on cardiac imaging and is often found in association with other cardiac defects with ventricular septal defect as the commonest. It presents a diagnostic challenge on transthoracic echocardiography in coexistence with ventricular septal defect in adulthood, mimicking other cardiac defects. Unruptured sinus of valsalva aneurysm coexisting with ventricular septal defect usually presents with symptoms of rupture—a surgical emergency in the third and fourth decade of life. We report a case of a 24-year-old single mother referred to the Cardiology Clinic of the University of Nigeria Teaching Hospital with 5 months symptoms of heart failure. Investigations showed cardiomegaly, prominent pulmonary conus, and upper lobe vessel diversion on chest x-ray. There were bigeminal premature ventricular contractions, left atrial abnormality, biventricular hypertrophy with a right strain on electrocardiogram, and bidirectional membranous ventricular septal defect with right sinus of valsalva aneurysm on transthoracic echocardiography. Keywords: Aneurysm, bidirectional, sinus of valsalva, unruptured, ventricular septal defect
How to cite this article:
Udora NC, Ojiakor AG, Oguanobi NI, Anakwue RC. Unruptured sinus of valsalva aneurysm coexisting with ventricular septal defect. Int J Med Health Dev 2023;28:188-92 |
How to cite this URL:
Udora NC, Ojiakor AG, Oguanobi NI, Anakwue RC. Unruptured sinus of valsalva aneurysm coexisting with ventricular septal defect. Int J Med Health Dev [serial online] 2023 [cited 2023 Jun 3];28:188-92. Available from: https://www.ijmhdev.com/text.asp?2023/28/2/188/372153 |
| Key Messages | |  |
Sinus of valsalva aneurysm in isolation is relatively rare; usually, it coexists with a ventricular septal defect. In coexistence, both cardiac anomalies can mask the effect of the other or mimic other cardiac defects on transthoracic echocardiography.
| Introduction | | |
Sinus of valsalva aneurysm (SOVA) is an expansion of the aortic root between the aortic valve annulus and the sinotubular ridge.[1] It occurs due to a deficient or absent elastic fibers and muscle at the junction of the aortic media and the annulus fibrosus. Often, an aneurysmal SOVA is found in the right coronary sinus followed by the noncoronary and left coronary sinus.[1],[2]
The natural history of an unruptured SOVA is not known but can be complicated by aortic regurgitation (AR) in 30%–50% of patients.[1] The asymptomatic ones are usually unruptured, but in a few cases, dyspnea, palpitations, or exertional chest pain may occur. Other presentations include arrhythmia and complete heart block.[1],[2] Large SOVA can be a cardiac source of emboli mimicking ischemic heart disease when major coronary arteries are obstructed.[1] The most common complication and presentation of SOVA is rupture, which typically occurs between 20 and 40 years.[1] The speed with which a rupture occurs, its location, and the size determine the outcome. Symptoms include chest and abdominal pain, dyspnea, symptoms of acute heart failure, hemodynamic compromise, cardiac tamponade, and sudden cardiac death.[1]
We report a case of a 24-year-old single mother who was referred from a peripheral teaching hospital to the Cardiology Clinic of the University of Nigeria Teaching Hospital (UNTH) with features of heart failure and a bidirectional membranous ventricular septal defect associated with an aneurysmal right sinus of valsalva. A written informed consent was obtained from the patient to publish report and investigation images. The authors have no conflicts of interest in this case.
| Case History | | |
Miss CI—a 24-year-old woman—presented at the Cardiology Clinic of the UNTH with 5 months duration of chest pain, 3 months of shortness of breath, and generalized body swelling of 1 week. Symptoms were associated with weight loss, cough, paroxysmal nocturnal dyspnea, orthopnea, palpitation, dizziness, and a darkish discoloration of lips, palms, and soles. She developed worsening easy fatiguability, generalized body itch and yellowish discoloration of the eyes with a high-grade intermittent fever 1 week prior to presentation. Her childhood and pregnancy history were uneventful. She had no personal or family history of hypertension, diabetes mellitus, or heart disease. She does not have sickle cell disease, has no allergies, and does not take herbal medications.
Examination findings showed a young woman in respiratory distress, cyanosed with a bilateral pitting leg edema up to the knees. Her pulse rate was 102 beats per minute, small volume, and regular with a blood pressure of 138/60 mmHg. Jugular venous pulsation was elevated, and there was an active precordium with a diffuse apex located at the 7th left intercostal space, anterior axillary line. Heart sounds I, II, III were present with a grade 4/6 pansystolic murmur located maximally at the left mid-sternal border. There was a loud, nonsplitting pulmonary component of the second heart sound.
Respiratory rate was 35 cycles per minute, and there were bibasal fine crepitations.
The abdomen was distended but moved with respiration, with the right hypochondrial tenderness. There was a tender hepatomegaly with the liver measuring 6 cm below the right costal margin, midclavicular line. Ascites was demonstrable by shifting dullness.
Chest x-ray showed cardiomegaly with a prominent pulmonary conus and upper lobe vessel diversion [Figure 1]. | Figure 1: Chest x-ray showing cardiomegaly, prominent pulmonary conus, and upper lobe vessel diversion
Click here to view |
Electrocardiogram [Figure 2] showed sinus rhythm with premature ventricular contractions occurring in bigeminy, left atrial abnormality, and biventricular hypertrophy with a strain pattern on the right. | Figure 2: Electrocardiogram showing premature ventricular contractions in bigeminy, left atrial abnormality, and biventricular hypertrophy with a strain pattern on the right
Click here to view |
Echocardiography showed all heart chamber dilatation with a trileaflet aortic valve and an aneurysmal right coronary cusp with some areas of calcification. There was grade 2 AR on color (maximum velocity = 400 cm/s, pressure gradient = 64 mmHg), a functional grade 2 tricuspid regurgitation on color (maximum velocity = 264 cm/s, pressure gradient = 27 mmHg), and an estimated right ventricular systolic pressure of 42 mmHg. A flattened interventricular septum was noted throughout the cardiac cycle. Perimembranous ventricular septal defect (VSD) with a shunt size of 1.1 cm and an interval shunt from the left to right ventricle were also noted. Other findings include a nonsignificant atrial septal aneurysm and a dilated pulmonary trunk (5.0 cm). The systolic and diastolic functions of both ventricles were normal. The inferior vena cava was dilated (2.3 cm) with less than 50% collapsibility on deep inspiration.
The conclusion was coexisting right SOVA and bidirectional perimembranous VSD with moderate pulmonary hypertension [Figure 3], and [Figure 4]A and [Figure 4]B. | Figure 3: Parasternal short axis view (aortic level); arrow showing a right sinus of valsalva aneurysm. AO = aorta, LA = left atrium, PA = main pulmonary artery, RA = right atrium, RVOT = right ventricular outflow tract
Click here to view |
 | Figure 4: Modified apical five-chamber views. A, Ventricular septal defect measuring 1.1 cm; B, color envelope showing flow through the ventricular septal defect. AO = aorta, LA = left atrium, LV = left ventricle, RA = right atrium, RV = right ventricle
Click here to view |
Pertinent findings on blood and urine work up were an erythrocyte sedimentation rate of 110 mm/h, potassium level of 3.5 mmol/L, and a trace of protein in urine.
| Discussion | | |
SOVA is a relatively uncommon congenital or acquired heart defect found incidentally on cardiac imaging.[3] The incidence ranges from 0.1% to 3.5% of all congenital cardiac anomalies.[3],[4] Usually, it coexists with other cardiac anomalies of which VSD is the commonest in 30%–60% of cases; others include bicuspid aortic valve, aortic valve stenosis, pulmonary stenosis, tetralogy of fallot, coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and AR and tricuspid regurgitation.[3],[4],[5]
The coexistence of a membranous VSD and SOVA may be due to defect with the fusion of the two sides of the distal bulbous septa in utero.[3] Although supracristal/infundibular VSD is frequently associated with SOVA among the Asians, membranous VSD is the predominant type in the Westerners.[6] The two defects in coexistence may present challenges with diagnosing a VSD on transthoracic echocardiography because the aneurysm may overlap the VSD, and the left atrial and ventricular dilation may not be obvious.[3],[7] In the presence of a ruptured SOVA (RSOVA), a three-dimensional or transoesophageal echocardiography may be necessary as the VSD shunt may be submerged by the large RSOVA shunt.[3] They may also undergo independent pathological changes and variable presentations, but SOVA seems to present earlier with its rupture from literatures. A SOVA coexisting with a VSD will likely rupture, and the rupture point, shape, and size of the SOVA are affected.[7] The management of an asymptomatic unruptured SOVA is not clear, but in view of likely future complications, early correction by some authors has been recommended.[4],[8] However, in the presence of a VSD, a surgical procedure is indicated.
In conclusion, SOVA coexisting with a VSD requires a meticulous TTE using multiple views as misdiagnosis can easily occur.
Acknowledgement
We thank Prof. E. C. Ejim for helping with the echocardiographic technical issues in making the diagnosis.
Patient consent statement
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 sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Weinreich M, Yu PJ, Trost B Sinus of valsalva aneurysms: Review of the literature and an update on management. Clin Cardiol 2015;38:185-9.  |
| 2. | Udora NC, Ejim EC, Okoye JU Unruptured sinus of valsalva aneurysm, with dissection into the interventricular septum. Int J Med Health Dev 2017;22:150-5. |
| 3. | Sarhan HHT, Shawky AH, Anilkumar S, Elmaghraby A, Sivadasan PC, Omar AS, et al. Association of ruptured sinus of valsalva aneurysm and congenital ventricular septal defect: A case series. Eur Heart J Case Rep 2021;5:1-6. |
| 4. | Nezafati P, Nezafati MH, Hoseinikhah H Unruptured aneurysm of sinus of valsalva coexisting with the large ventricular septal defect and severe aortic regurgitation in a young man. Case Rep Med 2015;2015:396098. |
| 5. | Masaki N, Iwatsuka R, Nagahori W, Ohno M, Arakawa T, Suzuki M, et al. Three-dimensional echocardiography could distinguish a ventricular septal defect adjacent to asymptomatic ruptured sinus of valsalva aneurysm. J Cardiol 2008;51:139-43. |
| 6. | Kim HR, Kim SJ, Lim KH, Kim JM, Lee JH, Kim YG, et al. A case of perimembranous ventricular septal defect associated with sinus of valsalva aneurysm mimicking membranous septal aneurysm. J Cardiovasc Ultrasound 2015;23:113-7. |
| 7. | Jin Y, Han XM, Wang HS, Wang ZW, Fang MH, Yu Y, et al. Coexisting ventricular septal defect affects the features of ruptured sinus of valsalva aneurysms. Saudi Med J 2017;38:257-61. |
| 8. | Vural KM, Sener E, Taşdemir O, Bayazit K Approach to sinus of valsalva aneurysms: A review of 53 cases. Eur J Cardiothorac Surg 2001;20:71-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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