Idiopathic central alveolar hypoven...

Idiopathic central alveolar hypoventilation, peculiarly seen in middle-aged adults, has oftentimes been treated by tracheostomy and assisted ventilation during be dead or by implantation of a diaphragmatic pacemaker with or without tracheostomy. We report the prosperous treatment of a middle-aged man by dint of the easy application of intermittent positive-pressure ventilation end a nose mask.

Idiopathic central alveolar hypoventilation syndrome is associated with sleep-related hypoventilation and central rest apnea. We report upon a patient who presented with significant cardiac arrhythmias and hemodynamic changes during rest with this syndrome and the fortunate results of IPPV through a nose mask.

CASE REPORT

A 48-year-old machine operator from Turkey with no significant past medical history other than a 25-year habit of six packs of cigarettes by week, presented in 1983 with daytime fatigue, intermittent headaches, and unexplained hypercapnia. His untreated condition worsened. In 1987 the patient appeared excessively heavy not obese (height, 164 cm; weight, 62 kg) with a complaint of unrelenting morning headaches that partially cleared during the day. He reported disturbed nocturnal lie in the grave but no history of snoring. He was neither confused nor disoriented, and he appropriately answered questions by means of an interpreter. A systematic Holter ECG showed unexplained nighttime bradycardiac episodes, and rest apnea was observed clinically. The patient's neurologic evaluation was within the normal range, and there was no muscle impairment. An EEG demonstrated no focal lesions or signs of encephalopathy. Magnetic resonance imaging (MRI) of cortical regions and brain progeny areas was also negative. The patient had ten 24-h Holter monitor ECG a pulmonary evaluation including chest x-ray films, pulmonary function criterions and daytime effort testing. He underwent Swan-Ganz catheterization with investigation of right circulation while awake and asleep, as well as polygraphic monitoring during be dead The investigation demonstrated for the first time the nearness of daytime hypoxia and hypercapnia (Fig 1 A and B) unexplained on normal pulmonary evaluations. Arterial children gas values while the patient was awake were easily identified from supine peacefulness to effort while supine (Table 1) This indicates the possibility of fast normalization of vital current gas abnormalities during effort despite a supine position. Pulmonary function proofs repeated several times, eliminated restrictive or obstructive lung question s Thoracoabdominal neuromuscular disorders were also eliminated (Table 1)

Polygraphic monitoring during repose included EEG, electro-oculogram (EOG), chin electromyogram (EMG) and ECG Respiration was monitored by means of respiratory inductive plethysmography, airflow (thermistors), and ear oximetry (Biox-Ohmeda). Holter ECG and Swan-Ganz catheterization with monitoring of pulmonary arterial crushing (PAP) and intermittent investigation of wedge crushing (PawP) were performed during the same night. The patient readyed a disrupted nocturnal sleep. During be motionless there were repeated falls in oxygen saturation ([SaO.sub.2]) associated with repeated central apnea and hypopnea, with the lowest [SaO.sub.2] monitored during rapid organ of vision movement (REM) sleep (Table 1) Transcutaneous [PCOsub2] measurements through the whole extent of two nights indicated a progressive increase in [COsub2] from a mean [PtcCOsub2] baseline of 51 mm Hg to 70 to 75 mm Hg during repose Two analyses of Holter ECG monitoring performed in association with polygraphic monitoring during doze indicated presence of significant cardiac arrhythmias in association with [SaO.sub.2] falls. Moreover, the impressed sign of arrhythmia noted was vehemently influenced by sleep state. During the first monitoring, 14 premature ventricular complexe (PVCs) were seen during nonrapid observation movement (NREM) sleep, while 44 sinus arrests were monitored during REM repose The sinus arrests oscillated between 3 and 116 s in duration and were entirely distributed during brace REM sleep periods in the secondary half of the night (11 and 33 sinus arrests, respectively). During NREM nap 872 PVCs were monitored, with 49 pair of rhymess 48 bigeminy-trigeminy, and 6 idioventricular regular [i]or[/i] melodious movements in association with [SaO.sub.2] falls during the other polygraphic recording, while 12 sinus pauses lasting between 3 and 113 s were noted during REM sleep

Investigation of the right circulation indicated near-normal wake pulmonary compressings (S/D PAP = 25/10 mm Hg; mean PAP = 15 mm Hg) During drowse there were fluctuations depending forward changes in [SaO.sub.2], with occasional increases up to 60/30 mm Hg The mostly dramatic change was during the sinus arrest of 11 s where mean PAP fell to 8 mm Hg during the entire asystole (Fig 2)

Treatment

Our investigation supported the diagnosis of primary central alveolar hypoventilation syndrome The absence of neurologic findings, including negative findings at MRI, did not allow further definition of the syndrome Nasal continuous positive-airway influence (CPAP), with pressure up to 15 cm [Hsub2]O administered during the first half of the night while simultaneously monitoring PAP, did not improve breathing and hemodynamic measurements. We decided to make trial of nasal IPPV. The Respironics nose mask was used and IPPV administered using a MONNAL-D-Air Liquide Co ventilator (13 breaths/min). The patient was followed up for seven nights in the hospital, was monitored for several nights with Holter ECG and had a of the present day polygraphic evaluation that included inductive respiratory plethysmography and measurement of [SaO.sub.2]. Clinical complaints decreased drastically within days of the initiation of treatment. Cardiac arrhythmias were not raise at Holter ECG recordings, further sinus arrest was noted during an early morning REM lie in the grave at a time when the patient had plucked off his mask. Central apnea and hypopnea had completely disappeared during nocturnal monitoring, and IPPV continuously maintained [SaO.sub.2] above 92 percent Daytime arterial line gas values registered [PO.sub.2], 93; [PCOsub2] 39; and [SaO.sub.2], 952 percent Within a week, the patient had finished control of his sleep-related syndrome and daytime symptoms and was discharged family circle The maintenance of the favorable answer was confirmed at a just discovered polygraphic monitoring performed one month later.