MyMedR

Fulltext Emergency stenting of vertical vein in a neonate with obstructed supracardiac total anomalous pulmonary venous drainage

Lim WK, Wong MN, Tan SK

Med J Malaysia, 2014 Jun;69(3):138-9.

PMID: 25326356 MyJurnal

A late preterm newborn baby presented with respiratory distress and increasing cyanosis within 2 hours of birth. Bedside transthroracic echocardiography showed a critically obstructed vertical vein in a supracardiac total anomalous pulmonary venous drainage (TAPVd). Emergency stenting of the vertical vein was successfully performed at 24 hours of life.

Matched MeSH terms: Cyanosis

Congenital cyanotic heart disease with situs inversus totalis: an autopsy case report

Dayapala A, Kumar V

Am J Forensic Med Pathol, 2009 Jun;30(2):171-4.

PMID: 19465810 DOI: 10.1097/PAF.0b013e3181875a79

Sudden deaths because of congenital heart diseases are infrequently referred to the forensic pathologist for autopsy. Many of such deaths, if already diagnosed are released directly from the hospital without autopsy. Even forensic pathologists face a few difficulties in performing the autopsy on such infrequent cases, as they are not always updated with the anatomy of anomalies. While dealing with such cases, the concerned forensic pathologist is compelled to refer literature and textbooks again to understand the nature of developmental defects. This is especially so when dealing with cases of situs inversus accompanied by transposition of great arteries and other congenital cardiac abnormalities and variants such as single ventricle, double outlet right ventricle, Taussig-Bing variety etc. In the present case also, situs inversus with transposition of great vessels and other anomalies have been noted and studied.

Matched MeSH terms: Cyanosis/etiology; Cyanosis/pathology

Fulltext Cor triatriatum dexter: A rare cause of childhood cyanosis

Zainudin AR, Tiong KG, Mokhtar SA

Ann Pediatr Cardiol, 2012 Jan;5(1):92-4.

PMID: 22529613 DOI: 10.4103/0974-2069.93725

Cor triatriatum dexter is a rare congenital heart anomaly where the right atrium is divided into two chambers by a membrane. We report a boy who had persistent mild cyanosis and diagnosed to have cor triatriatum dexter with secundum atrial septal defect by transoesophageal echocardiography. Interestingly, he had persistent mild cyanosis despite insignificant obstruction to the right ventricular inflow and normal pulmonary artery pressure. The pathophysiology, approach to the diagnosis, and mode of treatment are also discussed.

Matched MeSH terms: Cyanosis

Fulltext Tell tale of tablets in bronchus

Shimpi T, Chawla A, Shikhare S

Med J Malaysia, 2015 Feb;70(1):36-7.

PMID: 26032528

Foreign body (FB) aspiration is an emergency of concern at all ages. However, in adults, it can present with atypical symptoms such as shortness of breath, wheezing or rarely cyanosis. Aspiration of oral medications is seen in the elderly population with impairment of protective airway mechanism. Treatment of choice is endoscopic removal of the foreign body. We report such a case of foreign body aspiration (potassium chloride tablet), diagnosed on imaging and subsequently developed bronchostenosis. There are a very few reported cases of oral potassium supplement aspiration and associated complications in the literature.

Matched MeSH terms: Cyanosis

Cortical hyperostosis, rare adverse effect of prostaglandin

Ng RL, Koay HS, Jamil MT

Med J Malaysia, 2020 11;75(6):748-749.

PMID: 33219191

We describe here an infant girl with ductal dependent complex cyanotic heart disease, who required prostaglandin infusion for a total of five months prior to Blalock-Taussig shunt procedure. Her alkaline phosphatase activity was raised after seven weeks being on prostaglandin and only dropped to the normal range seven days after discontinuing prostaglandin infusion. During our review at five months old, her limbs were grossly swollen and radiographic examination showed dense periosteal reaction in the long bones. Based on the clinical findings and investigations, she was diagnosed to have cortical hyperostosis, which is an uncommon side effect of prostaglandin. She underwent right Blalock-Taussig Shunt procedure successfully with no major complications. Unfortunately, she succumbed to infection two months after surgery.

Matched MeSH terms: Cyanosis

Fulltext Pertussis resurgence in children: a cross-sectional, single centre study in North-west Malaysia

Salwana Ku Md Saad, Karniza Khalid, Abdul Nasir Mohamed Abdul Kadher

Malaysian Journal of Paediatrics and Child Health, 2017;23(2):0-0.

MyJurnal

Introduction: Vaccine-preventable diseases such as pertussis are re-emerging in Malaysia during recent years.

Objective: This research aims to study the local incidence of clinical pertussis among paediatric patients admitted to Hospital Tuanku Fauziah, Perlis over two-year period.

Method: A cross-sectional study was designed in Department of Paediatrics, Hospital Tuanku Fauziah, Perlis from 1 January 2013 till 31 January 2015. Data were collected from medical and laboratory record of cases admitted for clinical pertussis. Analyses for descriptive data were done using frequency and percentage; Pearson chi-square or Fisher exact was used to test association.

Results: 81 cases of clinical pertussis were included in the study. Out of this, there were a total of 28 confirmed cases of pertussis. There was a steady increment in the incidence of pertussis throughout the study period. Cyanosis emerged as the only clinical sign significantly associated with pertussis (p = 0.011). Majority of the confirmed pertussis cases were too young to be immunized (n = 13, 46.4%).

Conclusion: Reappraisal of local health system to strengthen herd immunity in the community is warranted to control disease spread.

Matched MeSH terms: Cyanosis

Fulltext Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution

Lee J, Sivalingam S, Alwi M

Ann Pediatr Cardiol, 2017 9 21;10(3):281-283.

PMID: 28928615 DOI: 10.4103/apc.APC_168_16

We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation.

Matched MeSH terms: Cyanosis

Fulltext Single rare central lesion with triple common aerodigestive symptoms

Mohamad I, Mohamad IS, Nik Hassan N

Malays Fam Physician, 2018;13(1):57-58.

PMID: 29796215 MyJurnal

An elderly gentleman with a known history of
well-controlled hypertension presented with
a three-week history of hoarseness associated
with mild breathlessness. There was no episode
of cyanosis, no noisy breathing, and no
reduction in effort tolerance. There was also no
history of chest pain or orthopnea. He denied
any feeling of food stuck in his throat or chest,
and he had no history of choking sensations
during meals. He, however, was unable to
count from 1 to 10 in one breath, and lung
auscultation revealed reduced air entry on both
sides. A chest radiograph was then obtained. (Copied from article).

Matched MeSH terms: Cyanosis

Fulltext The blue man who presented with a stroke

Koh KC, Wong MH

Malays Fam Physician, 2011;6(1):29-31.

PMID: 25606217 MyJurnal

A 19-year-old man presented with sudden onset of right eye ptosis, diplopia and giddiness. He had no previous medical illnesses with negative history of exertional dyspnoea, epistaxis, haemoptysis, palpitations, chest pain and chronic cough. Examination revealed central cyanosis, digital clubbing, polycythaemia, partial ptosis of right eye, diplopia on right gaze and dilated right pupil. Examination of the chest revealed pectus excavatum but no cardiac murmurs were heard. Investigations revealed a solitary right pulmonary arteriovenous malformation with two feeder vessels which were successfully embolized surgically.

Matched MeSH terms: Cyanosis

Fulltext Jellyfish Envenomation Resulting In Vascular Insufficiency And Neurogenic Injury of Upper Limb

Choong C, Chan HZ, Faruk NA, Bea KC, Zulkiflee O

Malays Orthop J, 2015 Nov;9(3):49-51.

PMID: 28611910 MyJurnal DOI: 10.5704/MOJ.1511.007

Following a week after a jellyfish sting, a young man presented with regional cyanosis and threat of distal gangrene secondary to vascular spasm in the forearm. The patient also suffered from transient paresis and numbness of the affected upper limb. Contrasted imaging revealed unopacified vessels in the distal forearm and worsening swelling warranted emergency surgical fasciotomy for impending compartment syndrome. This case highlights the occurrence of jellyfish envenomation and the need for early treatment.

Matched MeSH terms: Cyanosis

Fulltext First reported case of Haemoglobin-M Hyde Park in a Malay family living in Malaysia

Loong TY, Chong DL, Jamal AR, Murad NA, Sabudin RZ, Fun LC

EXCLI J, 2016;15:630-635.

PMID: 28096792 DOI: 10.17179/excli2016-613

Haemoglobin (Hb)-M Hyde Park, also known as Hb-M Akita is a rare type of hereditary Hb M due to autosomal dominant mutation of CAC>TAC on codon 92 of β globin gene resulting in the replacement of histidine by tyrosine on β globin chain. This variant Hb has a tendency to form methaemoglobin (metHb). The iron ion in metHb is oxidized to ferric (Fe3+) which is unable to carry oxygen and the patients manifest as cyanosis clinically. A 9-year-old Malay girl was incidentally found to be cyanotic when she presented to a health clinic. Laboratory investigations revealed raised methaemoglobin levels and Hb analysis findings were consistent with Hb-M Hyde Park. β gene sequencing confirmed a point mutation of CAC>TAC on codon 92 in one of the β genes. The family study done on the individuals with cyanosis showed similar findings. A diagnosis of heterozygous Hb-M Hyde Park was made. Patients with this variant Hb usually presented with cyanosis with mild haemolysis and maybe misdiagnosed as congenital heart disease. No further treatment is needed as patients are relatively asymptomatic. Although the disease is harmless in the heterozygous carriers but the offspring of the carriers may suffer severe haemolytic anaemia when the offspring also inherit other β haemoglobinopathies/thalassemia. This can happen due to high prevalence of β thalassemia carrier (3.5-4 %) found in Malaysia. At the time of writing, this is the first case of hereditary Hb-M Hyde Park diagnosed in a Malay family living in Malaysia.

Matched MeSH terms: Cyanosis

Fulltext The mystery of 'saturation gap': a case of dapsone-induced methaemoglobinemia in a pregnant mother with leprosy

Tang ASO, Yeo ST, Teh YC, Kho WM, Chew LP, Muniandy P

Oxf Med Case Reports, 2019 Jan;2019(1):omy111.

PMID: 30697428 DOI: 10.1093/omcr/omy111

Limited data regarding methemoglobinemia in pregnancy, particularly secondary to dapsone is available up to date. We report a case of dapsone-induced methemoglobinemia in a pregnant mother with multibacillary leprosy who presented with fever, productive cough and cyanosis of 2 days duration 2 weeks after multidrug therapy was commenced. On examination, she had central cyanosis with low oxygen saturation (SpO2 = 84-88%). Arterial blood gas analysis showed PO2 of 111 mmHg and SO2 of 98 mmHg. Patient was administered 100% oxygen inhalation, but there was no improvement in cyanosis. Vitamin C (1000 mg/day) was prescribed. Dapsone was replaced by ofloxacin 200 mg twice daily. There was a gradual increase in SpO2 level. She delivered a healthy baby. In conclusion, clinicians should be aware of the side effects of dapsone and know how to promptly manage any undesirable events. Ofloxacin is a safe and feasible alternative in replacement of dapsone in pregnancy.

Matched MeSH terms: Cyanosis

Persistent valve of systemic venous sinus: a cause of neonatal cyanosis

Qureshi AU, Latiff HA, Sivalingam S

Cardiol Young, 2014 Aug;24(4):756-9.

PMID: 24016801 DOI: 10.1017/S1047951113001200

Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

Matched MeSH terms: Cyanosis/etiology*

Congenital nasal cavity stenosis in children with craniosyntosis: A report of 4 rare cases

Liew YT, Soo SS, Nathan AM, Manuel AM

Auris Nasus Larynx, 2017 Oct;44(5):635-638.

PMID: 27793496 DOI: 10.1016/j.anl.2016.10.001

Congenital bony nasal stenosis (CBNS) is a very rare but life-threatening cause of airway obstruction in neonates and infants. This review aims to assess the presentation and early airway management of 4 new cases of craniosynostosis with bilateral nasal cavity stenosis. Patients were treated with endoscopic endonasal widening of the nasal cavity and stenting. All patients were extubated well post-operatively with resolution of symptoms. They remained asymptomatic with stents in situ for at least 6 months with no complications reported. Minimally invasive endoscopic endonasal widening of the nasal cavity with stenting is an effective and safe way of addressing nasal cavity stenosis.

Matched MeSH terms: Cyanosis/etiology

Fulltext Pulmonary arteriovenous malformation: a rare cause of cyanosis in a child

Sharifah AI, Jasvinder K, Rus AA

Singapore Med J, 2009 Apr;50(4):e127-9.

PMID: 19421665

Pulmonary arteriovenous malformations are rare vascular anomalies. We report a 12-year-old girl who presented with exertional dyspnoea, cyanosis and clubbing since the age of five years, and multiple pulmonary arteriovenous malformations. Computed tomography pulmonary angiogram showed a large pulmonary arteriovenous malformation at the lower lobe of the right lung. Pulmonary angiogram showed a large right lung arteriovenous malformation and two small left lung arteriovenous malformations. The multiple arteriovenous malformations were occluded with Gianturco coils. She is now asymptomatic and on regular follow-up.

Matched MeSH terms: Cyanosis/etiology*

An Unusual Type of Totally Anomalous Pulmonary Venous Connection to the Superior Cavoatrial Junction

Shamsuddin AM, Wong AR, Anderson RH, Corno AF

World J Pediatr Congenit Heart Surg, 2016 Jul;7(4):490-3.

PMID: 26795906 DOI: 10.1177/2150135115603330

A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pulmonary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months.

Matched MeSH terms: Cyanosis

Fulltext An uncommon life-threatening cause of electric alternans with sudden onset of breathlessness and chest pain

Benjamin Ng Han Sim

Borneo Journal of Medical Sciences, 2020;14(2):0-0.

MyJurnal

Phasic ECG voltage changes or electrical alternans is a well-described ECG changes seen in the pericardial effusion and cardiac tamponade. Popular as once believed, this ECG features are no longer considered pathognomonic for pericardial effusion and cardiac tamponade. Electric alternans is observed in pneumothorax especially left-sided pneumothorax. This is a case of a 41-year-old man who presented with chest pain and breathlessness to the emergency department. Assessment in the emergency unit revealed an obvious distress man with a respiratory rate of 60 breaths/min with cyanosis There were generalised rhonchi and prolonged expiratory breath sound appreciated. Chest X-ray (CXR) was done and diagnosed to have left tension pneumothorax. Initial electrocardiogram (ECG) showed electrical alternans in all leads. He was intubated for respiratory distress followed by chest tube insertion. His initial ECG findings resolved after treatment of the tension pneumothorax. Doctors need to evaluate the cardiac findings along with respiratory findings.

Matched MeSH terms: Cyanosis

A Case of Paraganglioma with Cyanotic Congenital Heart Disease

Wahab NA, Chien BH, Omar MR, Aziz AA, Mustafa N, Sukor N, et al.

Acta Med Indones, 2021 Jan;53(1):77-81.

PMID: 33818409

Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life.

Matched MeSH terms: Cyanosis

Fulltext Birth prevalence and late diagnosis of critical congenital heart disease: A population-based study from a middle-income country

Mat Bah MN, Sapian MH, Alias EY

Ann Pediatr Cardiol, 2020 09 17;13(4):320-326.

PMID: 33311920 DOI: 10.4103/apc.APC_35_20

Aims: There are limited data regarding critical congenital heart disease (CCHD) from middle-income countries (MIC). This study aims to determine the birth prevalence, rate of late diagnosis, and influence of timing of diagnosis on the outcome of CCHD.

Setting and Design: Retrospective observational cohort study in the State of Johor, Malaysia.

Subjects and Methods: All infants born between January 2006 and December 2015 with a diagnosis of CCHD, defined as infants with duct-dependent lesions or cyanotic heart disease who may die without early intervention. The late diagnosis was defined as a diagnosis of CCHD after 3 days of age.

Results: Congenital heart disease was diagnosed in 3557 of 531,904 live-born infants and were critical in 668 (18.7%). Of 668, 347 (52%) had duct-dependent pulmonary circulation. The birth prevalence of CCHD was 1.26 (95% confidence interval: 1.16-1.35) per 1000 live births, with no significant increase over time. The median age of diagnosis was 4 days (Q1 1, Q3 26), with 61 (9.1%) detected prenatally, and 342 (51.2%) detected late. The highest rate of late diagnosis was observed in coarctation of the aorta with a rate of 74%. Trend analysis shows a statistically significant reduction of late diagnosis and a significant increase in prenatal detection. However, Cox regression analysis shows the timing of diagnosis does not affect the outcome of CCHD.

Conclusions: Due to limited resources in the MIC, the late diagnosis of CCHD is high but does not affect the outcome. Nevertheless, the timing of diagnosis has improved over time.

Matched MeSH terms: Cyanosis

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