Recurrent cyanotic episodes with severe arterial hypoxaemia and intrapulmonary shunting: a mechanism for sudden death.

Southall, D P; Samuels, Martin; Talbert, D.G.

doi:10.1136/adc.65.9.953

Cited by 93 publications

(45 citation statements)

References 26 publications

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“…None of these infants (cases or controls) had a structural congenital heart lesion that could have produced a right to left shunt and resultant ventilation/perfusion mismatch. An intermittent intrapulmonary right to left shunt was, therefore, considered to be the most likely cause of these hypoxaemic episodes.5 13 The analysis of breathing patterns during prolonged desaturations performed in the present study confirms our previous observation that the potential to develop a mismatch between ventilation and perfusion may also be present in clinically well preterm infants.…”

Section: Discussionsupporting

confidence: 88%

Oxygen saturation and breathing patterns in infancy. 2: Preterm infants at discharge from special care.

Poets

Stebbens

Alexander

et al. 1991

Archives of Disease in Childhood

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Overnight 12 hour tape recordings of arterial oxygen saturation (SaO2, pulse oximeter in the beat to beat mode), breathing movements, and airflow were made on 66 preterm infants (median gestational age 34 weeks, range 25-36) who had reached term (37 weeks) and were ready for discharge from the special care baby unit. No infant was given additional inspired oxygen during the study. The median baseline SaO2 was 99-4% (range 88-9-100% In the present study, the same techniques have been applied to a group of preterm infants who were studied when they were ready for discharge from the special care baby unit and had reached at least 37 weeks' gestation. Two specific questions were asked: what is the influence of gestational age on the pattern of oxygenation, and (based on our study of term infants4) how does oxygenation differ between full term and preterm infants?Patients and methods Between August 1986 and July 1987 all preterm infants who were born at three maternity hospitals and who received special care immediately after birth had overnight tape recordings made of selected physiological variables. The study was carried out when infants were assessed as ready for discharge, and only on those who were considered to have a good prognosis for survival. A total of 305 infants fulfilled the criteria, and 261 (86%) had parental consent for the investigation. The project was approved by the hospitals' ethics committees. For the purpose of this study, all 72 infants with gestational ages of 37 weeks or more at the time of discharge, and who underwent their recordings within the three days before discharge, were included in the analysis.All 72 infants underwent 12 hour overnight recordings of arterial oxygen saturation (SaO2) (Nellcor N100 with new software equivalent to N200 and specially modified to provide beat to beat data); each photoplethysmographic pulse waveform from a pulse oximeter (for the validation of the saturation signals); breathing movements from a volume expansion capsule (Graseby) or from respiratory inductance plethysmography (Studley Data Systems); and nasal airflow either through a thermistor (Yellow Springs Instruments) or from expired carbon dioxide sampling (Engstrom Eliza). The recordings were stored on tape (Racal FM4) and subsequently printed onto graph paper by an ink jet recorder at 3-2 mm/second. As the study was prospective and non-invasive the recordings were not used for clinical management.Six recordings (8%) gave poor quality oxygen saturation signals throughout and were removed from the study. Of the remaining 66 recordings, 31 (47%) were done on the last day before discharge, 26 (39%) two days, and eight (12%)

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Section: Discussionsupporting

confidence: 88%

Oxygen saturation and breathing patterns in infancy. 2: Preterm infants at discharge from special care.

Poets

Stebbens

Alexander

et al. 1991

Archives of Disease in Childhood

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“…In DiMario's (3) prospective study, as many as four of 95 children had learning disablilities and are in special education classes, although there was no proof that BHS were the cause of these disabilities. One of the patients reported by Southall et al (12) presented with progressive cerebral atrophy shown by computed tomography, although neuropathology was not reported following sudden death.…”

Section: Figure 1) a Rhythm Strip From An Event Recorder At The Time mentioning

confidence: 94%

“…Unexpected death has been reported in children with BHS, although other causes of death were entertained in some cases (10,11). In 1990, Southall et al (12) reported sudden death in eight of 51 infants and young children with recurrent episodes of cyanosis and loss of consciousness precipitated by crying. Autopsies suggested other causes of death in six children, but sudden death could have been precipitated by primary breath-holding in at least two children.…”

Section: Figure 1) a Rhythm Strip From An Event Recorder At The Time mentioning

confidence: 99%

“…It is imperative to rule out other causes of loss of consciousness and seizures in breath-holders. Multiple medications have been tried with variable success, including iron therapy for children with low hemoglobin levels (18), piracetam (19), and central sympathetic modulators such as tetrabenzamine and clonidine for cyanotic BHS (12). Because bradycardia and asystole, especially in pallid BHS, are most likely mediated by excessive parasympathetic tone, anticholinergic treatment with atropine (20) or transdermal scopolamine (21) has been attempted with inconsistent results.…”

Section: Figure 1) a Rhythm Strip From An Event Recorder At The Time mentioning

confidence: 99%

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A pacemaker for asystole in breath-holding spells

Legge

Kantoch

Seshia

et al. 2002

Paediatrics &Amp; Child Health

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Two cases of young children with frequent severe breath-holding spells complicated by prolonged asystole and seizures are reported. A ventricular pacemaker was implanted in each child, and both have subsequently remained free of syncope, although they continue to exhibit breath-holding behaviour.Key Words: Asystole; Breath-holding; Pacemaker; Syncope Un stimulateur en cas d'asystolie dans les spasmes du sanglotOn rend compte du cas de deux jeunes enfants présentant des spasmes du sanglot fréquents et graves, compliqués par une asystolie prolongée et des convulsions. Un stimulateur ventriculaire a été implanté chez chaque enfant, et si tous deux ont continué à faire des spasmes du sanglot, ils n'ont plus fait de syncopes. B reath-holding spells (BHS) are common in early childhood, typically occurring between the ages of six months and four years. Severe pallid spells and, less commonly, cyanotic spells may be complicated by the loss of consciousness, posturing and generalized seizures. A toddler and an infant who experienced prolonged asystole at the time of severe BHS and who were treated with the implantation of ventricular pacemakers are described. CASE 1 PRESENTATIONAn 18-month-old "determined and busy" girl was referred to the cardiology clinic with a history of syncope since the age of 11 months. All events were precipitated by the child being upset, usually when something was taken away from her without warning or when she experienced a minor injury. The child would cry hard; become cyanotic; stop breathing on expiration; turn silent, pale and limp; and lose consciousness. She would recover with an inspiratory gasp within a few minutes. The parents documented 49 BHS. On four occasions, the child developed opisthotonic posturing and myoclonic jerks. Twice the pupils became dilated and she became incontinent. Her tone and skin colour improved gradually over several minutes. Spells were followed by at least one-and-a-half hours of sleep. When the parents learned about asystole associated with breath-holding, they treated the individual episodes with oxygen, res-

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“…The presence of bilateral ptosis and unilateral miosis, neuroparalytic corneal ulceration and reduced pupillary light reflexes of the right eye indicated oculosympathetic paresis 7,8 , in the absence of spaceoccupying lesion of brain, neck and thorax.…”

Section: Case Historymentioning

confidence: 99%

Multiple pituitary hormone abnormalities, fever, behavioral problems, seizures and apnoic spells in a 6-year old girl

Karavanaki

Divoli²,

Dattani³

et al. 2002

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A 6-year old girl was examined having two years previously presented a transient Cushings syndrome, followed by recurrent hyponatremia, attributed to inappropriate ADH secretion (SIADH). The brain MRI showed no abnormalities on repeated examinations, except for a suggestion of empty sella syndrome. During the past two years she also presented recurrent episodes of a prolonged febrile illness of unknown origin. All investigations related to infectious, autoimmune neoplastic diseases, histiocytosis-X or neurosarcoidosis were negative and the fever was characterized as central. The patient also presented episodes of tonic-clonic seizures, myoclonias and behavioral problems (alternating states of irritability, sleepiness and apathy, optic and hearing illusions and phobias) with or without hyponatremia. Her cerebrospinal fluid (CSF) examination was not indicative of encephalitis and the encephalogram (EEG) showed only slowing of background activity. At the age of 4.75 years she, started to have recurrent episodes of hypopnoea/apnoea with severe desaturation and hypercapnia, occasionally requiring intubation and ventilation. She also developed unilateral miosis corneal ulceration and bilateral ptosis (oculo-sympathetic paresis). Repeat brain MRI and CT scans of the mediastinum excluded organic causes of apnoeas and of oculosympathetic paresis, such as neuroblastoma or lymphoma. Furthermore, on a 24hour electrocardiogram recording, using power spectral analysis, significantly reduced heart rate variability was observed, by comparison with age-specific normal ranges. Thus the apnoeas, ptosis, miosis and temperature instability were attributed to autonomic dysfunction. During the same period, the patient presented significant growth retardation and growth hormone (GH) deficiency was confirmed during two provocative tests (peak GH levels: 3.1 and 2.9 ng/ml (normal>10). Moreover, thyrotropin (TSH) deficiency and persistent hyperprolactinemia were detected. She was started on hGH and thyroxine. She was also put on fluid restriction and increased sodium intake for her SIADH. She was advised to use O 2 administration by mask in case of apnoeas. The child died at age 6 6/12 years as a

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Recurrent cyanotic episodes with severe arterial hypoxaemia and intrapulmonary shunting: a mechanism for sudden death.

Cited by 93 publications

References 26 publications

Oxygen saturation and breathing patterns in infancy. 2: Preterm infants at discharge from special care.

Oxygen saturation and breathing patterns in infancy. 2: Preterm infants at discharge from special care.

A pacemaker for asystole in breath-holding spells

Multiple pituitary hormone abnormalities, fever, behavioral problems, seizures and apnoic spells in a 6-year old girl

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