During COVID pandemic, a 2-year-old child affected by steroid-resistant nephrotic syndrome (genetic diagnosis still in progress), ESRD in peritoneal dialysis for 1 year and autoimmune hypocortisolism in glucocorticoid replacement therapy, was admitted to our Paediatric Unit for rapidly worsening severe HT in the past 2 months.
Recent past medical history was characterised by a fast-solving episode of severe acute respiratory syndrome and heart failure without any proved infective underlying cause; the episode occurred one month before admission and solved within 48 h. The subsequent echocardiography showed: left ventricle hypertrophy with a mild septal dyskinesia, left ejection function (EF) of 52% and persistence of dilation of left coronary vessels stable since previous control, all features compatible with a hypertensive heart disease.
The patient underwent surgical nephrectomy because of persistent severe HT, resistant to multiple antihypertensive oral drugs, including ACE inhibitors, angiotensin receptor blockers, alpha-agonist, beta-blockers and calcium channel blocker (CCBs), and fenoldopam parenteral therapy. After surgery, antibiotic therapy was started and peritoneal dialysis continued regularly. Blood pressure control progressively improved. The antihypertensive therapy i.v. was discontinued 10 days after surgery; blood pressure was controlled with triple oral therapy with alpha-agonist, beta-blockers and CCBs.
During surgery-day, the mother resulted positive for SARS-CoV2 at molecular test. The day after surgery, the patient himself tested positive at nasopharyngeal swab, even if asymptomatic.
Three days after surgery and 2 days after his first positive swab, the patient experienced fever, rhinitis, sore throat, vomiting, diarrhoea and abdominal pain.
Five days after the first positive swab, fever became not responsive to antipyretic therapy and C-reactive protein markedly increased. Abdominal ultrasound was negative for post-surgical complications. Hydrocortisone replacement therapy was increased. On day six, the patient experienced dyspnoea and hypoxia with lung crackles and worsening hepatomegaly. Chest x-ray showed findings of interstitial pneumonia (“extended and shaded thickening mainly parahilar”). Initially ventilation support with high flow nasal cannula (HFNC) was started. However, one day later, a rapid clinical worsening occurred with severe dyspnoea and oxygen desaturation not responsive to oxygen therapy and maximisation of ventilation support with HFNC, associated with hypotension and tachycardia. Bedside echocardiography revealed left ventricle ejection function (LVEF) of 11–15%, moderate mitral regurgitation and dilation of left heart with right ventricle pressed, with no cardiac or pleural effusion.
Patient required admission to the paediatric intensive care unit (PICU), invasive ventilation support and inotropic support therapy with milrinone. Antihypertensive therapy was stopped.
Lab tests demonstrated: anaemia (Hb 8.1 g/L), lymphocytopenia (500 cells/mmc), mild thrombocytopenia (82,000 cells/mmc), elevated acute inflammation reactants (PCR 110 mg/dL, ferritin 1611 μg/L, D-dimer 714 μg/L, IL2-R 7294 KU/L, IL6 93 ng/L), increase of heart failure lab-index NT-proBNP >175,000 ng/L with mild increase of troponin 4.7 ng/L; abnormal liver function tests (AST 242 UI/L, ALT 176 UI/L) and increased LDH (665 U/L). The peripheral blood smear was negative for schistocytes. Abdominal ultrasonography revealed liver non homogeneous echo-pattern and periportal oedema.
On suspicion of SARS-CoV2 related disease, since the first day of PICU admission, we performed: steroid therapy (methylprednisolone 2mg/kg/day i.v. once daily for five doses), azithromycin for three days, heparin i.v. (10 UI/kg/h c.i.) and hyperimmune plasma (100 mg/kg/die—three doses). The patient also required blood and albumin transfusions. Peritoneal dialysis was maximalised from 10 h/day to continuous dialytic treatment. Dextrose concentration of dialytic fluid and dwell time were modulated according to patient need. Cytokine filter hemodalysis was evaluated but not performed due to great need for filter priming with hemodynamically unstable patient.
After 24 h of mechanical ventilation, the echocardiography revealed fast improvement of heart function with LVEF increased to 45%. Five days after intubation, milrinone was discontinued and antihypertensive therapy was restarted. The patient remained intubated for three more days, then was supported with non invasive ventilation for 24 h and finally with HFNC up to 7 days after extubation.
Finally, the patient experienced a progressive clinical improvement, HT was controlled by oral therapy, respiratory failure resolved and hepatomegaly reduced. After 18 days since the first positive swab and after 9 days since intubation, nasopharyngeal swab resulted negative for SARS-CoV2. Two weeks after intubation NT-proBNP decreased and echocardiography revealed a recovery of previous heart function.