Advertisement

COVID-19 as a confounding factor in a child submitted to staged surgical palliation of hypoplastic left heart syndrome: One of the first reports of SARS-CoV-2 infection in patients with congenital heart disease

Open ArchivePublished:June 05, 2020DOI:https://doi.org/10.1016/j.jtcvs.2020.05.081

      Graphical abstract

      COVID-19 could be a confounding factor for the diagnosis and management of patients with CHD. SARS-CoV-2 infection can progress with significant hypoxemia and mimics surgical complications.
      See Commentaries on pages e103, e104 and e105.
      In December 2019, in the province of Wuhan, China, authorities identified an outbreak of pneumonia caused by a novel coronavirus, colloquially referred at COVID-19. The virus has now spread worldwide, reaching pandemic proportions.
      • World Health Organization
      Pneumonia of unknown cause—China.
      Although adult patients with cardiovascular comorbidities are at increased risk of progressing with the severe form of the disease,
      • Zhou F.
      • Yu T.
      • Du R.
      • Fan G.
      • Liu Y.
      • Liu Z.
      • et al.
      Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study.
      it is not known whether the same holds true for children with congenital heart disease (CHD). Furthermore, both the incidence of infection and severity of disease seem to be much lower in children than in adults.
      • Lu X.
      • Zhang L.
      • Du H.
      • Zhang J.
      • Li Y.Y.
      • Qu J.
      • et al.
      SARS-CoV-2 infection in children.
      Here we describe the diagnosis and management of COVID-19 in a child with staged surgical palliation of hypoplastic left heart syndrome (HLHS). We believe this to be among the first reported cases of COVID-19 in a patient with CHD. This report was approved by the Research Ethics Committee of the Hospital Beneficência Portuguesa de São Paulo, and written informed consent was obtained from the patient's parents.

      Case Description

      On March 24, 2020, a 35-month-old girl diagnosed with HLHS underwent a Fontan procedure with an 18-mm polytetrafluoroethylene tube and tricuspid valve repair (comissural plasty). The patient demonstrated complete atrioventricular block after cross-clamp removal, necessitating temporary atrioventricular pacing, but regained a conducted sinus rhythm before leaving the operating room. In the intensive care unit (ICU), the patient was extubated and received metamizole and anticoagulants (heparin and enoxaparin) daily on the subsequent days, as part of routine postoperative care.
      Her oxygen saturation worsened the day following the operation, with values <60% (Figure 1) with the onset of dyspnea and dry cough on postoperative day (POD) 2 (Figure 2). Echocardiography demonstrated low flow in the Fontan circulation, raising the suspicion of thrombosis in the Fontan circuit. Cardiac catherization was performed, demonstrating no evidence of thrombosis and a patent fenestration. A left pleural effusion was noted, and a left pleural drain was placed to evacuate it, without a significant improvement in oxygen saturation or tachypnea.
      Figure thumbnail gr1
      Figure 1Respiratory frequency and oxygen saturation on physical examination. Blue lines represent the minimum and red lines, the maximum respiratory frequency; green lines represent the minimum and yellow lines, the maximum oxygen saturation.
      Figure thumbnail gr2
      Figure 2Historical timeline of the procedures and symptoms. On postoperative day (POD) 2, the patient began to show symptoms of COVID-19, with cough followed by hypoxemia, which necessitated orotracheal intubation on POD 5. After pacemaker implantation for atrioventricular block, the patient presented with new symptoms (fatigue, vomiting, and abdominal discomfort) and was submitted to high-flow oxygen therapy. She subsequently showed improvement and was discharged from the intensive care unit on POD 20. ICU, Intensive care unit.
      On POD 5, the patient began to experience intermittent complete atrioventricular block necessitating pacing. On POD 7, a permanent epicardial dual-chamber pacemaker system was implanted, positioned in the right atrium and right ventricle and with the generator in the upper abdominal wall. Following the surgery, the patient progressed with worsening hypoxemia, with O2 saturation dropping as low as 37%. Arterial blood gas tests showed a decrease in blood pH and inversion between O2 and CO2 partial pressures (Figure 3).
      Figure thumbnail gr3
      Figure 3Arterial blood gases and blood pH. On postoperative day (POD) 7, the patient presented worsening of the hypoxemia, maintaining low blood pH and inversion between O2 and CO2 partial pressures.
      On POD 8, the patient began to have abdominal discomfort, followed the next day by respiratory distress and rare vomiting episodes (Figure 2). Milrinone, diuretics, and sildenafil were prescribed. A new chest radiograph showed multiple pulmonary consolidations, most evident on the upper third of the right lung (Figure 4, A). Laboratory tests revealed neutrophilic leukocytosis (leukocytes: 14,090 cells/mm3; neutrophils: 10,470 cells/mm3; lymphocytes: 2560 cells/mm3; Figure 5) and a C-reactive protein (CRP) value of 5.45 mg/L, which increased up to 11.36 mg/L on POD 12. Vancomycin and cefepime were initiated on POD 9.
      Figure thumbnail gr4
      Figure 4Chest radiography. A, On postoperative day (POD) 8, evidencing multiple pulmonary consolidations. B, On POD 16, showing a sole remaining opacity in the upper third of the right lung.
      Figure thumbnail gr5
      Figure 5Main outcomes and implications of the case. A, Historical timeline demonstrating the onset of COVID-19 symptoms following the surgical procedure. B, Chest radiographs from postoperative days 8 and 16, showing the recovery of pulmonary consolidations. C, Take-home messages from the case. ICU, Intensive care unit; CHD, congenital heart disease.
      The patient progressed with the worsening of cough on POD 10. On this day, the patient's mother reported that she herself had anosmia, raising the concern that the child could possibly have COVID-19. Both parents reported flu-like symptoms starting a week before the date of the patient's surgery. The patient was isolated, and a nasopharyngeal swab specimen was obtained and sent for the detection of viral respiratory pathogens.
      On POD 11, the patient progressed with new onset of respiratory distress and worsening of hypoxemia, and high-flow O2 was added to her respiratory support. The intervention was successful in stabilizing her clinical parameters, and her general status improved over the next several days. On POD 13, reverse-transcription polymerase chain reaction of the nasopharyngeal swab confirmed COVID-19. The patient was removed from high-flow O2 on POD 14 and maintained on oxygen nebulization. On POD 16, the patient continued to exhibit dry cough, but a chest radiograph showed reduction of the pulmonary consolidations with a sole remaining opacity in the upper third of the right lung (Figure 4, B).
      Over the next several days, the patient improved. She was transferred from the ICU to the ward on POD 20, and from there to home on POD 24.

      Discussion

      The present report, among the first to describe COVID-19 in a child with CHD, brings some interesting considerations (Figure 5). First, it highlights the importance of considering, during a pandemic time, COVID-19 as a possible cause of complications in children with CHD, notably those recently submitted to surgical procedures. In these cases, it is commonly assumed that most complications are directly associated with the disease or the procedure itself.
      • Ghosh R.M.
      • Griffis H.M.
      • Glatz A.C.
      • Rome J.J.
      • Smith C.L.
      • Gillespie M.J.
      • et al.
      Prevalence and cause of early Fontan complications: does the lymphatic circulation play a role?.
      However, particularly in complex cyanotic CHD, COVID-19 can progress with significant hypoxemia and mimic stenosis, kinks or thrombotic events. These finding could be also described for other pneumonia, but considering the present situation, COVID-19 should be one of the first diagnostic hypothesis. Still, in the present case, COVID-19 did not greatly modify the outcomes, with the exception of prolonged ICU stay.
      Second, it is crucial to understand how prescribed drugs may play a role in response to the infection. In this regard, at least 3 interactions must be highlighted in this case. The first involves the use of analgesic drugs with antipyretic effects, herein represented by metamizole but is also applicable to paracetamol, ibuprofen, and others. Such drugs could mask fever and delay infection diagnosis. On the other hand, it might contribute to prevent further worsening of the pneumonia. The second, the use of anticoagulants, is associated with decreased mortality in severe COVID-19 patients.
      • Tang N.
      • Bai H.
      • Chen X.
      • Gong J.
      • Li D.
      • Sun Z.
      Anticoagulant treatment is associated with decreased mortality in severe coronavirus disease 2019 patients with coagulopathy.
      In this regard, the subject patient was receiving anticoagulants, which could also have contributed to preventing the development of more serious disease manifestations.
      Third, testing patients before surgery may play an important role in cases like the one reported here. The preoperative testing of children undergoing surgery for CHD has been proposed and has become standard in many services.
      • Levy E.
      • Blumenthal J.
      • Chiotos K.
      • Dearani J.A.
      COVID-19 FAQs in pediatric cardiac surgery. World J Ped Cong Heart Surg.
      ,
      • Stephens E.H.
      • Dearani J.A.
      • Guleserian K.J.
      • Overman D.M.
      • Tweddell J.S.
      • Backer C.L.
      • et al.
      COVID-19: crisis management in congenital heart surgery.
      Still, it might not be advisable or practical in all settings. In Brazil, for instance, where there is a queue of thousands of tests awaiting analysis by a limited number of laboratories, results are being released only slowly. If we opted to preoperatively test all children undergoing surgery for CHD, this prolonged waiting time could be detrimental for most of them. If any preoperative testing is performed, the use of polymerase chain reaction–based testing of respiratory secretions is recommended. Another recommendation is to screen parents entering the hospital or clinic for both COVID-19 symptoms and contact with known positive cases.
      • Levy E.
      • Blumenthal J.
      • Chiotos K.
      • Dearani J.A.
      COVID-19 FAQs in pediatric cardiac surgery. World J Ped Cong Heart Surg.
      Despite these and other recently published recommendations, there are still no consensus guidelines for this emerging situation.
      Finally, this case reinforces the importance of parents, siblings, and caregivers of children with CHD to practice primary infection control prevention and engage in quarantine and social distancing measures. These measures, particularly critical for all CHD patients, become even more relevant if the patient is expected to undergo a surgical procedure.

      References

        • World Health Organization
        Pneumonia of unknown cause—China.
        (Available at:)
        • Zhou F.
        • Yu T.
        • Du R.
        • Fan G.
        • Liu Y.
        • Liu Z.
        • et al.
        Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study.
        Lancet. 2020; 395: 1054-1062
        • Lu X.
        • Zhang L.
        • Du H.
        • Zhang J.
        • Li Y.Y.
        • Qu J.
        • et al.
        SARS-CoV-2 infection in children.
        N Engl J Med. 2020; 382: 1663-1665
        • Ghosh R.M.
        • Griffis H.M.
        • Glatz A.C.
        • Rome J.J.
        • Smith C.L.
        • Gillespie M.J.
        • et al.
        Prevalence and cause of early Fontan complications: does the lymphatic circulation play a role?.
        J Am Heart Assoc. 2020; 9: e015318
        • Tang N.
        • Bai H.
        • Chen X.
        • Gong J.
        • Li D.
        • Sun Z.
        Anticoagulant treatment is associated with decreased mortality in severe coronavirus disease 2019 patients with coagulopathy.
        J Thromb Haemost. 2020; 18: 1094-1099
        • Levy E.
        • Blumenthal J.
        • Chiotos K.
        • Dearani J.A.
        COVID-19 FAQs in pediatric cardiac surgery. World J Ped Cong Heart Surg.
        Date: April 21, 2020
        ([Epub ahead of print])
        • Stephens E.H.
        • Dearani J.A.
        • Guleserian K.J.
        • Overman D.M.
        • Tweddell J.S.
        • Backer C.L.
        • et al.
        COVID-19: crisis management in congenital heart surgery.
        Ann Thorac Surg. April 11, 2020; ([Epub ahead of print])

      Linked Article

      • Commentary: “Murder on the Orient Express of pandemic: COVID was found guilty, but was it the murderer?”
        The Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 2
        • Preview
          In Agatha Christie's novel Murder on the Orient Express, detective Hercule Poirot brilliantly resolves the murder of Mr Ratchett, a kidnapper, and proposes 2 possible solutions. The first is a plausible solution that fits with the scenario and saves the unfortunate group of murderers, who were looking for revenge. The second solution describes what happened but would have sent all of them to jail. The investigators adopted the first solution as the truth to save the entire family from prison.
        • Full-Text
        • PDF
        Open Archive
      • Commentary: Adapting the practice of congenital heart surgery to the coronavirus disease 2019 (COVID-19) pandemic
        The Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 2
        • Preview
          Bezerra and colleagues1 report on an interesting case from São Paulo, Brazil, where, as of this writing in June 2020, coronavirus disease 2019 (COVID-19) is raging. This worldwide pandemic took clinicians by surprise and, as a consequence, there is a lot to catch up on, such as the different manifestations of this new disease, how it affects patients with congenital heart disease (CHD), and on how to prevent it. Writing from the one-time epicenter of the COVID-19 crisis in NYC, our team has unfortunately been confronted with patients with CHD infected with COVID-19 at different perioperative time periods, such as in asymptomatic patients coming from home for semielective surgery who tested positive on their polymerase chain reaction (PCR) nasal swab test, in a patient with cardiomyopathy on extracorporeal membrane oxygenation support who required a ventricular assist device while still positive, and in postoperative patients after Fontan and after truncus arteriosus repair (after testing negative on their preoperative PCR tests).
        • Full-Text
        • PDF
        Open Archive
      • Commentary: The importance of operative timing in the era of coronavirus disease 2019 (COVID-19)
        The Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 2
        • Preview
          The current worldwide coronavirus disease 2019 (COVID-19) pandemic has required all health care practitioners to consider the care of their patients in an entirely new light. This is especially paramount when determining the timing for any medical procedure, as providers must now, more than ever, appreciate the importance of critically evaluating what procedures are truly urgent and cannot be deferred to an elective basis. Despite the initial focus of the COVID-19 pandemic being directed toward adult patients, pediatric patients have also experienced a significant burden of this novel disease,1,2 and patients with congenital heart disease are certainly not exempt.
        • Full-Text
        • PDF
        Open Archive