Ascending aortic dissecting aneurysm with rupture into the right atrium associated with COVID-19D Kamanakeri, A Anoop, S Harikrishnan, A Gopalakrishnan
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.jpgm_903_21
Source of Support: None, Conflict of Interest: None
The coronavirus disease 2019 (COVID-19) pandemic era has seen a change in the observed incidence and presentations of aortic dissections across the globe. New York witnessed a precipitous drop in the monthly surgical volumes of acute type A aortic dissection in the early months after the outbreak of the pandemic. However, after the initial lull, the number of acute aortic cases have returned to pre-COVID-19 pandemic levels in Europe, the US, and Asia. The marked surges in the number of at-home deaths and the increase in the number of chronic aortic dissections in the COVID-19 pandemic era present a matter of major concern. While a dual diagnosis of type A aortic dissection with COVID-19 is potentially catastrophic, we present a case where the symptoms of the latter likely precipitated the former.
A 42-year-old man presented with a history of low-grade fever for one day and acute onset of breathlessness, orthopnea, and diaphoresis following a bout of dry cough. He had undergone mechanical aortic valve replacement (#27 TTK Chitra) 16 years ago for severe aortic regurgitation; and, single chamber (VVI) pacemaker implantation for complete heart block ten years ago. Histopathology of the excised aortic valve had revealed a rheumatic process in the aortic valve and fusion of the right and non-coronary cusps consistent with a bicuspid aortic valve.
On examination, he was dyspneic and agitated. He was 168 cm tall, weighed 68 kg, and had no features of Marfan's syndrome or other connective tissue diseases. The oxygen saturation on room air was 94% (upright) and 85% (supine). He was afebrile (temperature 98.8°F), pulse rate was 70/minute, and all peripheral pulses were palpable. His blood pressure was 98/60 mmHg in the right upper limb. Jugular venous pressure was elevated till the angle of the mandible with cannon waves. Cardiovascular examination suggested cardiomegaly, with markedly displaced right heart border. The first heart sound was normal. The aortic prosthetic valve closing click was audible, and the pulmonary component of the second sound was loud. A grade 2 mid-systolic murmur was heard at the aortic area. No diastolic murmur could be appreciated. Respiratory system examination revealed decreased breath sounds in the right hemithorax. There were no adventitial sounds.
Chest X-ray revealed gross cardiomegaly with the right heart border abutting the right thoracic border, dilated ascending aorta, and blunted right costophrenic angle [Figure 1]a. The right bronchus appeared compressed and displaced caudally. The left lung shadows were normal. Electrocardiography showed right ventricular paced rhythm at 70 beats/minute [Figure 1b]. The response of the pacemaker to the placement of a magnet was normal (100 beats/minute).
Transthoracic echocardiography revealed an aneurysmal ascending aorta with spontaneous echo contrast and a normally functioning competent tilting disc aortic prosthetic valve. A 3 mm defect with the continuous flow from the ascending aorta into a dilated right atrium was imaged [Video 1][Additional file 1]. Urgent computed tomography (CT) of the chest and abdomen revealed dissection flaps in the dilated ascending aorta (130 mm) with rupture into the right atrium [Figure 2] and [Figure 3]. The right mainstem bronchus was compressed by the aortic aneurysm with a collapse of the right upper lobe. There was mild right pleural effusion but no pericardial effusion. The patient's COVID-19 rapid antigen test was reported positive. There were no CT features of pneumonia or lung involvement secondary to COVID-19.
Laboratory investigations revealed Hb 16.5 g/dl, WBC count of 7500/cu.mm with 48% polymorphs, 46% lymphocytes and 6% eosinophils and platelet count was 2.62 lakhs/ cu.mm. Other blood investigations were: lactate dehydrogenase 400 U/L, creatinine 2.29 mg/ dL, troponin - t quantitative 0.16 ng/ml, N-terminal pro-brain natriuretic peptide 7430 pg/ml, and D-dimer 2230 micro gm/L.
The patient developed worsening respiratory distress with metabolic acidosis shortly after that and was mechanically ventilated with inotropic support. While arranging for emergent aortic root replacement, he developed ventricular fibrillation with refractory cardiogenic shock and could not be revived. He died within two hours of arrival at the emergency. COVID-19 RT-PCR also tested positive, though the report came after the patient had died.
Acute aortic dissections have been noted to have seasonal variations even in the pre-COVID-19 pandemic era. Type A dissections tend to cluster in the winter months and the influenza season and have higher in-hospital mortality rates. The International Registry of Acute Aortic Dissection demonstrated a higher incidence of acute aortic dissection in winter months that was independent of climate, suggesting the role of concomitant environmental factors in the pathogenesis.
Respiratory infections like influenza and whooping cough are known to precipitate acute mechanical cardiovascular events. Both influenza and COVID-19 appear to impose similar effects on the cardiovascular system., Cytokine storm and consequent vascular inflammation occur in both influenza and coronavirus infections like SARS-CoV-2 and MERS-CoV. COVID-19 induces a T-helper-1 mediated cell response through increased production of pro-inflammatory cytokines in the serum (IL1B, IFNγ, IP10, and MCP1). Respiratory distress in coronavirus infection is also associated with surges in IL-1, IL-6, and IL-12 levels. Circulating IL-6 is associated with increased aortic dimensions in genetic aortopathies. The bicuspid aortic valve is often associated with aortopathy and ascending aortic dilatation. The vigorous cough in the index patient with aortopathy could have acted as the trigger for the catastrophe in the background of elevated pro-inflammatory cytokines. Unfortunately, the patient died soon after admission, and these pro-inflammatory markers could not be obtained. Aortic dissection tends to rupture most commonly into the pericardial space. Rupture into the right atrium with the formation of a fistula is rare.
Although direct causative evidence is lacking, similar reports from other investigators suggest the possibility of an association between aortic dissection and COVID-19. Dual diagnosis of type A aortic dissection and COVID-19 was associated with extremely complicated postoperative course and high mortality. This is in stark contrast to the outcomes of surgery for aortic dissection in those who tested negative for COVID-19 in the pandemic era, which is not dissimilar to the pre-pandemic era. According to the consensus statement by the Indian Association of Cardiovascular and Thoracic Surgery (IACTS), acute type A aortic dissections should be operated upon as an emergency in the COVID-19 pandemic era; in line with the recommendations of the American College of Surgeons. However, acquisition of a COVID-19 test is recommended regardless of the symptoms in all cases of acute aortic dissections in the pandemic era, while the management continues to be guided by hemodynamic stability with consideration for urgent surgical repair. The present patient was critically ill at presentation with rapid deterioration of hemodynamics before surgery could be offered.
Aorto-right atrial fistula formation is a rare complication of aortic dissection. Acute type A aortic dissections complicated by COVID-19 infection constitute a deadly conundrum with high fatality rates. Vigorous cough associated with COVID-19 can trigger catastrophic aortic dissection and rupture. Although the prognosis is guarded, a patient with the dual diagnosis of type A aortic dissection with associated COVID-19 should be considered for emergency high-risk surgery.
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[Figure 1], [Figure 2], [Figure 3]