Cause Of Hypertension In Children Research Articles

7439 Mercury toxicity presenting as Pheochromocytoma

Abstract Disclosure: A. Ansar: None. K. McNerney: None. B.A. Marshall: None. Background: Mercury intoxication is a rare cause of hypertension in children and can mimic other endocrinopathies such as pheochromocytoma. Mercury affects catecholamine metabolism and can cause elevated catecholamine levels, thereby incorrectly suggesting a catecholamine-secreting tumor. Clinical Case: An 8-year-old girl presented to the ED with progressive night sweats, intractable migratory myalgias, hypertension and tachycardia. Patient was in her usual state of health until 2 weeks before presentation when she started to have worsening intermittent pains in her chest, arms, back, and knees. She also experienced restlessness and inability to sleep. On presentation she was hypertensive, with blood pressure of 146/116 mm Hg while calm, heart rate 125 bpm, afebrile. She was anxious, irritable, and seemed uncomfortable. Her skin was dry and rough with a scaly eczematous rash on the arms and legs. She had persistently elevated blood pressure in upper and lower extremities, unremarkable echocardiogram and electrocardiogram, and a normal result on fundoscopic examination. Her initial electrolytes, creatinine, and urinalysis were all normal and remained so on serial evaluations. Urine drug screen was negative. Thyroid function panel showed normal TSH with slightly elevated Free T4 2.28 ng/dL (0.9-1.7), but a normal Free T3 3.9 pg/mL (2.0-4.4) and Free T4 by equilibrium dialysis 1.8 ng/dL (0.8 - 2.0). CT of the chest, abdomen and pelvis was negative and did not show any lesions. Plasma renin activity was 4.6 ng/mL/hour, supine, (<2.0) and plasma aldosterone level < 4.0 ng/dL (< 40 supine) were normal. 24-hour urinary norepinephrine was 90 μg/24h (13-65), epinephrine 37 μg/24h (0.2-10), urine metanephrines 195 μg/24h (18-144), urine normetanephrines 305 μg/24h (29-145), all elevated. Further history taking revealed that patient was taking Ayurvedic supplements prepared by hand by medicinal personnel in India for 3 months prior to presentation. These were stopped 2 weeks prior to her presentation, when she first became symptomatic. Her blood mercury level was 11 ng/mL (n <9), and urinary mercury excretion 67 μg/24 hours (n<10), both obtained 3 weeks after stopping supplementation. She was started on oral chelation therapy with succimer 10 mg/kg/day. Her hypertension was controlled with doxazosin. One week after initiation of therapy, her symptoms improved, and she became normotensive. Her acrodynia and irritability resolved. Urine metanephrine levels will be repeated at upcoming clinic follow up. Conclusion: Mercury toxicity should be considered in the differential of pheochromocytoma to avoid extensive diagnostic testing and morbidity associated with it. Lead, mercury, and arsenic have been detected in traditionally prepared Ayurvedic supplements. A careful history of intake of supplements can be important in cases with unusual symptomatology. Presentation: 6/3/2024

CARDIOVASCULAR OUTCOMES IMPROVE IN CHILDREN WITH RENOVASCULAR HYPERTENSION FOLLOWING ENDOVASCULAR AND SURGICAL INTERVENTIONS

Objective: Renovascular hypertension (RVH) is a common cause of hypertension in children. It is caused by renal artery stenosis reducing the blood supply to the renal parenchyma and activating the renin-angiotensin-aldosterone axis, often leading to cardiac remodelling. This is a longitudinal observational study that aims to describe the occurrence of hypertensive changes and their severity and also any improvement in cardiac remodelling after successful endovascular and/or surgical intervention in children with RVH. Design and method: All patients with RVH referred to our centre, who had received at least one endovascular intervention, were included. Data was collected by retrospective database review over a 22-year period. We assessed oscillometric blood pressure as well as eight echocardiographic parameters pre- and post-intervention: intraventricular septal diameter (IVSD), posterior wall diameter (PWD), left ventricular internal diastolic diameter (LVIDD), fractional shortening (FS), Relative Wall Thickness (RWT), Left Ventricular Mass (LVM) and Left Ventricular Ejection Fraction (LVEF). Results: 152 patients met inclusion criteria and had on average two endovascular interventions. Six children presented in frank heart failure. 54.4% achieved normal blood pressure (BP) control after intervention. Average z-scores improved in IVSD, PWD and FS; LVMI and RWT also improved. PWD saw the greatest reduction in mean difference in children with an abnormal (z-score reduction of 0.25, p<0.001) and severely abnormal (z-score reduction of 0.23, p<0.001) z-scores between pre- and post-intervention echocardiograms. 45.9% of children had a reduction in prescribed antihypertensive medications. Conclusions: Our study reports improvement in cardiac outcomes after either endovascular or surgical intervention. This is evidenced on BP control and echocardiogram changes in which almost half achieved normalisation in systolic BP readings and reduction in the number of children with abnormal echocardiographic parameters.

Cardiovascular outcomes improve in children with renovascular hypertension following endovascular and surgical interventions

BackgroundRenovascular hypertension (RenoVH) is a cause of hypertension in children. A common cause of RenoVH is renal artery stenosis which acts by reducing blood supply to renal parenchyma and activating the renin–angiotensin–aldosterone axis, often leading to cardiac remodelling. This longitudinal observational study aims to describe occurrence of cardiovascular changes secondary to RenoVH and also any improvement in cardiac remodelling after successful endovascular and/or surgical intervention.MethodsAll patients with RenoVH referred to our centre, who received ≥ 1 endovascular intervention (some had also undergone surgical interventions) were included. Data were collected by retrospective database review over a 22-year period. We assessed oscillometric blood pressure and eight echocardiographic parameters pre- and post-intervention.ResultsOne hundred fifty-two patients met inclusion criteria and had on average two endovascular interventions; of these children, six presented in heart failure. Blood pressure (BP) control was achieved by 54.4% of patients post-intervention. Average z-scores improved in interventricular septal thickness in diastole (IVSD), posterior Wall thickness in diastole (PWD) and fractional shortening (FS); left ventricular mass index (LVMI) and relative wall thickness (RWT) also improved. PWD saw the greatest reduction in mean difference in children with abnormal (z-score reduction 0.25, p < 0.001) and severely abnormal (z-score reduction 0.23, p < 0.001) z-scores between pre- and post-intervention echocardiograms. Almost half (45.9%) had reduction in prescribed antihypertensive medications, and 21.3% could discontinue all antihypertensive therapy.ConclusionsOur study reports improvement in cardiac outcomes after endovascular + / − surgical interventions. This is evidenced by BP control, and echocardiogram changes in which almost half achieved normalisation in systolic BP readings and reduction in the number of children with abnormal echocardiographic parameters.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information.Supplementary information

The enigma of primary hypertension in childhood.

Beginning in the 1970s, hypertension in children and adolescents has been defined as systolic and/or diastolic blood pressure (BP) that is equal to or greater than the 95th percentile of the normal BP distribution in healthy children. The definition of hypertension in adults is based on longitudinal data that links a BP level with an increased risk for subsequent adverse outcomes related to hypertension including heart failure, kidney failure, stroke, or death. The statistical definition of hypertension continues to be used in childhood because there have been no data that link a BP level in childhood with a heightened risk for adverse outcomes in adulthood. Findings from clinical and epidemiologic research have advanced understanding of high BP in childhood. While hypertension in some children can be secondary to underlying kidney, cardiovascular, or endocrine disorder, it is now known that primary (essential) hypertension can be present in childhood. The prevalence of hypertension in childhood is approximately 2-5% and another 13-18% of children and adolescents have elevated BP and are at heightened risk for developing hypertension. The leading cause of childhood hypertension is primary hypertension, especially in adolescents. For children and adolescents with secondary hypertension, the treatment can focus on managing the underlying cause of hypertension. Less is known about managing primary hypertension in childhood, including diagnosis, evaluation, treatment, and possibilities for prevention. The phenotype of primary hypertension in childhood and recent findings will be discussed.

Primary aldosteronism caused by a pI157S somatic KCNJ5 mutation in a black adolescent female with aldosterone-producing adenoma.

Aldosterone-producing adenoma is a rare cause of hypertension in children. Only a limited number of cases of aldosterone-producing adenomas with somatic KCNJ5 gene mutations have been described in children. Blacks are particularly more susceptible to developing long-standing cardiovascular effects of aldosterone-induced severe hypertension. Somatic CACNA1D gene mutations are particularly more prevalent in black males whereas KCNJ5 gene mutations are most frequently present in black females. We present here a novel somatic KCNJ5 p.I157S mutation in an aldosterone-producing adenoma from a 16-year-old black female whose severe drug-resistant hypertension significantly improved following unilateral adrenalectomy. Prompt diagnosis of aldosterone-producing adenoma and early identification of gene mutation would enable appropriate therapy and significantly reduce cardiovascular sequelae.

Validation of computed tomography angiography as a complementary test in the assessment of renal artery stenosis: a comparison with digital subtraction angiography.

Renal artery stenosis is an important cause of hypertension in children, accounting for 5-10% of cases. When suspected, noninvasive imaging options include ultrasound (US), computed tomography (CT) angiography and magnetic resonance (MR) angiography. However, digital subtraction angiography (DSA) remains the gold standard. To investigate the accuracy and inter-reader reliability of CT angiography in children with suspected renal artery stenosis. This is a retrospective study of patients suspected of having renal artery stenosis evaluated by both CT angiography and DSA between 2008 and 2019 at a tertiary pediatric hospital. Only children who underwent CT angiography within 6months before DSA were included. CT angiography studies were individually reviewed by two pediatric radiologists, blinded to clinical data, other studies and each other's evaluation, to determine the presence of stenosis at the main renal artery and 2nd- and 3rd-order branches. The sensitivity, specificity and accuracy were calculated using DSA as the reference. The effective radiation dose for CT angiography and DSA was also calculated. Kappa statistics were used to assess inter-reader agreement. Seventy-four renal units were evaluated (18 girls, 19 boys). The patients' median age was 8years (range: 1-21years). Overall, CT angiography was effective in detecting renal artery stenosis with a sensitivity of 85.7%, specificity of 91.5% and accuracy of 88.9%. There was moderate inter-reader agreement at the main renal artery level (k=0.73) and almost perfect inter-reader agreement at the 2nd/3rd order (k=0.98). However, the sensitivity at the 2nd- and 3rd-order level was lower (14.3%). CT angiography provided excellent negative predictive value for evaluating renal artery stenosis at the main renal artery level (90.1%) and at the 2nd- or 3rd-order branches (82.7%). The median effective dose of CT angiography studies was 2.2mSv (range: 0.6-6.3) while the effective dose of DSA was 13.7mSv. CT angiography has high sensitivity and specificity at the main renal artery level with a lower radiation dose than previously assumed. Therefore, it can be used as a diagnostic tool in patients with low to medium risk of renal artery stenosis, and as a screening and treatment planning tool in patients at high risk.

The clinical and radiological cerebrovascular abnormalities associated with renovascular hypertension in children: a systematic review.

Renovascular disease is an important secondary cause of hypertension in childhood. In this cohort, many may have undiagnosed cerebrovascular disease, and some children present acutely with cerebrovascular complications. However, these associations are yet to be defined in the literature.A systematic review of clinical and radiological abnormalities associated with renovascular hypertension in the global pediatric (< 18 years) population. The MEDLINE, Embase, and Google Scholar databases were searched, from database inception to 26 January 2021. Primary articles were unrestricted by study design and geographical location but were limited to those published in English.A total of 303 individuals (median age: 7.6 years [range 10 days-17.9 years]; M:F, 174:129) from 34 studies were included, across 13 countries. Twenty-seven individual cases were published for children with coexisting renovascular hypertension and cerebrovascular disease. Most children had bilateral renal artery stenosis, secondary to fibromuscular dysplasia and had coexisting occlusive cerebrovascular disease. The majority presented with neurological symptoms, and cerebral complication ranged from asymptomatic cerebrovascular stenosis to acute stroke and posterior reversible encephalopathy syndrome. The location or underlying etiology of the renovascular disease did not predict the location or extent of the cerebrovascular disease. The evidence from the cohort studies was limited, as none specifically established a cohort of children with coexisting disease. Furthermore, the conclusions drawn were subjected to considerable bias from the treating clinicians.A prospective cohort of children with renovascular hypertension and cerebrovascular complications should be established so the long-term prognosis and impact of treatment may be better understood.

A rare cause of childhood hypertension detected in a school screening program: Answers.

A rare cause of childhood hypertension detected in a school screening program: Answers.

A rare cause of childhood hypertension detected in a school screening program: Questions.

A rare cause of childhood hypertension detected in a school screening program: Questions.

P1806OUTCOMES OF PEDIATRIC RENOVASCULAR HYPERTENSON: A SINGLE-CENTER EXPERIENCE

Abstract Background and Aims Renovascular disease is rare but important treatable cause of secondary hypertension in children. We aimed to evaluate the clinical presentations and long-term outcomes of pediatric patients with renovascular hypertension (RVH). Method We retrospectively reviewed medical records of patients with renovascular disease at our center between 1994 and 2019. Clinical courses including status of hypertension control with preservation of renal function during follow up were evaluated. Results 20 patients were diagnosed with RVH. 50 % (n = 10) were male, and median age at diagnosis was 10.1 (range 1.3 – 17.2) years, and median follow up period was 8.7 (range 0.1 – 24.6) years. 50 % (n = 10) presented with incidently detected high blood pressure (8 patients without symptoms, one with headache, and the other one with proteinuria), 25 % (n = 5) first admitted due to heart failure symptoms, and the rest (25 %, n = 5) presented with neurologic symptoms including seizure or paraplegia. Majority had no underlying disease except for 3 patients with Moyamoya disease. 80 % (n = 16) had unilateral renovascular stenosis. All patients showed elevated basal random renin activity (median 20.0, range 2.5 – 62.1 ng/ml/hr), and 45 % (n = 9) patients showed elevated basal random aldosterone level (median 822, range 266 – 2440 pg/ml). All patients needed antihypertensive medications for blood pressure control; 35 % (n = 7) of patients gained good control of blood pressure only with antihypertensive agents including angiotensin converting enzyme inhibitor/angiotensin receptor blocker (ACEI/ARB), 40 % (n = 8) of patients who underwent percutaneous transluminal angioplasty all still needed antihypertensive agents including ACEI for blood pressure control. 20 % (n = 4) of the patients initially showed profoundly low relative function of involved kidney on diuretic scan, leading to nephrectomy. Three of these patients with nephrectomy successfully discontinued all antihypertensive agent gaining good control of blood pressure. The remaining one patient showed progressive deterioration of relative function on the involved side of kidney during 13 years, ended up with nephrectomy, but couldn’t discontinue ACEI. Glomerular filtration rate (GFR) was within normal range for all patients at diagnosis. For patients without nephrectomy, mean relative function of the involved kidney on diuretic scan was 33.5 ± 11.4 % at diagnosis. There was no significant change or deterioration of relative renal function during a mean follow up period of 10 ± 8 (median 11.5, range 0 – 19.5) years, although they all used ACEI/ARB. All patients including patients with nephrectomy showed normal GFR with a mean of 114.1 ± 19.5 ml/min/1.73 m2 at the last follow up. Conclusion Antihypertensive medications including ACEI and ARB were safely used with no further deterioration of the renal function of the involved side with or without angioplasty. Pediatric RVH is well managed with preserved renal function in long-term follow up.

Long-Term Results after Diversion Surgery in Extrahepatic Portal Vein Obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery. Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery. Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery (p = 0.0015) as well as prothrombin activity (p = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt (p = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them. Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes.

Management and Outcomes of Childhood Renal Artery Stenosis and Middle Aortic Syndrome.

Renal artery stenosis (RAS) in isolation or in conjunction with middle aortic syndrome (MAS) are important vascular causes of childhood hypertension. Few longitudinal studies have assessed the risk of surgical or endovascular intervention, and outcomes by etiology or extent of vascular disease. In a retrospective study of 93 children seen over 30 years with RAS and/or MAS, data on vascular involvement (isolated RAS vs. RAS with MAS), etiology (unknown, inflammatory, or genetic), and management were collected. Time to first intervention (endovascular or surgical) was assessed by Cox regression. Mixed-effects analysis examined the longitudinal change in blood pressure after intervention compared to antihypertensive medications alone. Children were 7.0 ± 5.4 years old. Etiology was unknown in 50%, genetic in 26% and inflammatory in 24% of children. Children had isolated RAS (49%) or MAS with or without RAS (51%). Overall, 70% were managed with surgical or endovascular intervention. After adjusting for age, sex, and systolic blood pressure, children with unknown etiology had a higher risk of intervention compared to those with genetic and inflammatory diseases (hazard ratio 3.1, 95% confidence interval [CI] 1.7, 5.6). Children with RAS and MAS were less likely to receive intervention (hazard ratio 0.4, 95% CI 0.2, 0.8) than isolated RAS. Over a median follow-up of 2 years, 65% remained hypertensive. The longitudinal changes in systolic blood pressure did not differ by etiology, or between interventional and medical management. Hypertension persists despite endovascular or surgical management of childhood RAS and MAS highlighting the importance of close monitoring and ongoing medical management.

Genome-wide linkage and association study implicates the 10q26 region as a major genetic contributor to primary nonsyndromic vesicoureteric reflux

Vesicoureteric reflux (VUR) is the commonest urological anomaly in children. Despite treatment improvements, associated renal lesions – congenital dysplasia, acquired scarring or both – are a common cause of childhood hypertension and renal failure. Primary VUR is familial, with transmission rate and sibling risk both approaching 50%, and appears highly genetically heterogeneous. It is often associated with other developmental anomalies of the urinary tract, emphasising its etiology as a disorder of urogenital tract development. We conducted a genome-wide linkage and association study in three European populations to search for loci predisposing to VUR. Family-based association analysis of 1098 parent-affected-child trios and case/control association analysis of 1147 cases and 3789 controls did not reveal any compelling associations, but parametric linkage analysis of 460 families (1062 affected individuals) under a dominant model identified a single region, on 10q26, that showed strong linkage (HLOD = 4.90; ZLRLOD = 4.39) to VUR. The ~9Mb region contains 69 genes, including some good biological candidates. Resequencing this region in selected individuals did not clearly implicate any gene but FOXI2, FANK1 and GLRX3 remain candidates for further investigation. This, the largest genetic study of VUR to date, highlights the 10q26 region as a major genetic contributor to VUR in European populations.

Ultrasound imaging of renin-mediated hypertension.

The incidence of primary (i.e. essential) hypertension in children and adolescents is increasing; however, secondary hypertension, with an identifiable cause, remains relatively common and might be treatable or even curable. Renovascular hypertension is an uncommon but important secondary cause of hypertension in the pediatric population that can be associated with substantial morbidity. In this article we discuss renin-mediated causes of hypertension in children and related complications, review renal Doppler ultrasound techniques for the evaluation of renin-mediated hypertension (including both direct and indirect assessments), and briefly appraise the literature pertaining to renal Doppler ultrasound and the assessment of pediatric hypertension. Finally, we describe a proposed role for renal Doppler ultrasound in the workup of suspected renin-mediated hypertension in children and adolescents.

Sonographic evaluation of renovascular hypertension in the pediatric population: State-of-the-art.

Hypertension in children affects 2-5% of the pediatric population. Identifying secondary causes of hypertension is crucial, as some of these entities may be treatable. Renovascular hypertension accounts for 5-10% of the secondary causes of hypertension in children and is mainly related to fibromuscular dysplasia and neurofibromatosis type 1. Ultrasonographic evaluation of the kidneys, including Doppler interrogation, is the recommended primary screening tool. A comprehensive study of the upper abdomen and retroperitoneum allows for identification of additional underlying etiologies. The purpose of this review is to emphasize the different causes of renovascular hypertension in children one can diagnose during the initial ultrasonographic evaluation. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:282-292, 2017.

The importance of genetic counseling and genetic screening: a case report of a 16-year-old boy with resistant hypertension and severe hypokalemia

Liddle's syndrome, an autosomal dominant form of monogenic hypertension, is characterized by salt-sensitive hypertension with early penetrance, hypokalemia, metabolic alkalosis, suppression of plasma rennin activity and aldosterone secretion, and a clear-cut response to epithelial sodium channel blockers but not spironolactone therapy. Here, we describe the case of a 16-year-old boy patient with resistant hypertension (maintain 170–180/100–110 mm Hg after administration four kinds of antiypertensive drugs) and severe hypokalemia. After a series of checks, we exclude primary aldosteronism and renal artery stenosis and other diseases. Finally, the Liddle syndrome was diagnosed because of the DNA sequencing found that the proband's mother and himself had mutations P616L (c.1847 C>T) in the SCNN1B gene. Liddle syndrome should be considered as a cause of hypertension in children or adolescents particularly with suppressed renin activity. Early diagnosis and appropriately tailored treatment avoid complications of long-term unrecognized or inappropriately managed hypertension. Genetic testing has made it possible to make accurate diagnoses and develop tailored therapies for mutation carriers. The role of genetic testing and genetic counseling in establishing the early diagnosis of Liddle's syndrome is important.

Long-Term Outcomes of Pediatric Renovascular Hypertension

Background/Aims: Renovascular hypertension (RVHT) is an important cause of childhood hypertension. This study evaluated the clinical characteristics and outcomes of Korean children with RVHT. Methods: Children treated for RVHT between 2000 and 2015 at our center were retrospectively reviewed. Results: Forty-six children were followed for a median of 6.5 (0.66-27.23) years. Forty-five percutaneous transluminal angioplasties (PTAs) were performed in 32 children. At the last visit, clinical benefit was observed in 53.3% of children. Patients with comorbid cerebrovascular disease (CVD) showed less favorable long-term outcomes after PTA (clinical benefit in 41.7% vs. 61.1% in others) and higher restenosis rates (50% vs. 31.6% in others). Surgical procedures (bypass or nephrectomy) were performed in 8 patients. After surgery, blood pressure was normalized in 2 patients, improved in 3 patients, and unchanged in the remaining patients. Between PTA group (n=21) and medication group (n=14), percentage of atrophic kidneys became higher after follow-up period in medication group than in PTA group (60.0% vs. 26.1%, P=0.037). Conclusion: Aggressive treatment of pediatric RVHT yielded fair outcomes in our cohort. CVD comorbidity was associated with relatively poor PTA outcomes. To confirm our findings, larger cohort studies with a longer follow-up period are warranted.

Extra-Hepatic portal venous obstruction with portal biliopathy in infant presenting as neonatal cholestasis: a rare case report and review of literature

Extra-hepatic portal venous obstruction is most common cause of portal hypertension in children. Apart from variceal formation in gastric and esophageal mucosal layer, it has been postulated that external pressure and or ischemic injury play a role in portal biliopathy. We report an unusual case of a 6 month old infant who presented to us with clinical features suggestive of extra-hepatic biliary atresia; diagnosed as EHPVO with portal biliopathy, managed surgically.

Blocked mesocaval shunt with protein C deficiency in a child with extrahepatic portal hypertension

Portal vein thrombosis (PVT) is an important cause of extrahepatic portal hypertension in children. A majority of children with PVT of unknown etiology have functional protein C deficiency or abnormally elevated levels of anti-cardiolipin antibodies. We present an 8-year-old boy with portal hypertension with protein C deficiency, who underwent mesocaval shunt surgery due to recurrent episodes of bleeding esophageal varices and subsequently developed a block in the mesocaval shunt due to a thrombotic tendency.

Reversible Severe Pulmonary Hypertension after Adenotonsillectomy: A Case Report of a Child Treated at Bugando Medical Centre, Northwestern Tanzania.

Upper airway obstruction (UAO) due to adenotonsillar hypertrophy represents one of the rare causes of pulmonary hypertension in children. We report a case of adenotonsillar hypertrophy, managed at pediatric and otorhinolaryngology departments in Bugando Medical Centre (BMC), northwestern Tanzania, with complete remission of symptoms of pulmonary hypertension following adenotonsillectomy. A 17-month-old boy presented with difficulty breathing, dry cough, and noisy breathing since 1 year. He had facial and lower limb oedema with a pan systolic murmur at the tricuspid area, fine crepitations, and tender hepatomegaly. A grade II tonsillar hypertrophy and hypertrophied adenoids were seen on nasal and throat evaluation. A 2D-echocardiography showed grossly distended right atrium and ventricle, dilated pulmonary artery, and grade III tricuspid regurgitation. His final diagnosis was severe pulmonary hypertension with right-sided heart failure due to adenotonsillar hypertrophy. He had complete remission of cardiopulmonary symptoms after adenotonsillectomy and had normal control echocardiography six and twelve months after surgery. Children with symptoms of upper airway obstruction and cardiopulmonary involvement could benefit from routine screening for pulmonary hypertension. Adenotonsillectomy should be considered for possible complete remission of both UAO and cardiopulmonary symptoms.