Noninvasive Nasal Mask-Assisted Ventilation in Respiratory Failure of Duchenne Muscular Dystrophy

In both instances of VF, heart rate tended to increase gradually prior to the onset of the event. They differed in antecedent ST segment deviation and ectopic activity (ventricular tachycardia versus short cycle ectopic beats), the median time between onset of chest pain and interruption of sinus rhythm, and the propensity for evolving infarction. Necrosis in our patient occurred only after the second ischemic episode. A common denominator of both episodes of VF seemed to be active myocardial ischemia which can undoubtedly decrease the threshold of ventricular vulnerability We may speculate that our patient exhibited recurrent spastic occlusion of the left anterior descending coronary artery leading to consecutive episodes of subtotal and total occlusion of the artery episodes which were both critical enough to induce lethal ischemic arrhythmias. REFERENCES

Shea MJ, Deanfleld JE, Wilson R, DeLandsheere C, Jones l: Selwyn AE Transient ischemia in angina pectoris: frequent silent events with everyday activities. Am J Cardiol 1985; 56:34-38 2 De Servi S, Specchia G, Ardissino D, Falcone C, Mussini A, Angoli L, et aI. Angiographic demonstration of different pathogenetic mechanisms in patients with spontaneous and exertional angina associated with S-T segment depression. Am 1980; 45:1285-91

J Cardiol

3 Boden WE, Bough E~ Korr KS, Benham I, Cheorghiade M, Caputi A, et aI. Exercise-induced coronary spasm with S-T segment depression and normal coronary angiography Am J Cardioll981; 48:193-97 4 Yasue H, Omote S, Taldzawa A, Masao N, Hyon H, Nishida S, et ale Comparison of coronary arteriographic findings during angina pectoris associated with S-T elevation or depression. Am

J Cardioll981; 47:539-46 5 Previtali M, Klersy C, Salerno )A, Chimienti M, Panciroli C,

Marangoni E, et al. Ventricular tachyarrhythmias in Prinzmetals variant angina: clinical significance and relation to the degree and time course of S-T segment elevation. Am

J Cardioll983;

52:19-25 6 Mirvis OM, Ramanathan KB, Wilson JL. Regional blood flow correlates of ST segment depression in tachycardia-induced myocardial ischemia. Circulation 1986; 73:365-73 7 Gabliani GI, Winniford MD, Fulton KL, Hohnson SM, Mauritson DR, Hillis LD. Ventricular ectopic activity with spontaneous variant angina: frequency and relation to transient ST segment deviation. Am Heart J 1985; 110:40-43 8 Szlachic J, Waters DO, Miller D, Theroux E Ventricular arrhythmias during ergonovine-induced episodes of variant angina. Am Heart J 1984; 107:20-24 9 Nikolic C, Bishop RL, Singh JB. Sudden death recorded during Holter monitoring, Circulation 1982; 66:218-24 10 Maseri A, Severi S, De Nes M, L'Abbate A, Chierchia S, Marzilli M, et aI. Variant angina: one aspect of a continuous spectrum of vasospastic myocardial ischemia. Am J Cardio11978; 42:1019-35 11 Vincent G~I, Abildskov jA, Burgess MJ. Mechanisms of ischemic ST segment displacement. Circulation 1977; 56:559-66 1298

Noninvasive Nasal Mask-Assisted Ventilation In Respiratory Failure of Duchenne Muscular Dystrophy· David Segall, M.B., B.S., F.C.C.P.

The effects of noninvasive nasal mask-assisted ventilation were studied in two patients with chronic respiratory failure due to Duchenne's muscular dystrophy. Observations were made with continuous recordings of transcutaneous CO. and 0. and ear oximetry. In one case, the mean tcPco. fell from 72 mm Hg to 43 mm Hg. The tcPo. increased from 38 mm Hg to 62 mm Hg without supplementary oxygen. In the second case, hypercapnia associated with supplementary oxygen was corrected, and at five months' follow-up, hypoxemia was corrected without supplementary oxygen. Pr0longed therapy during sleep has resulted in sustained clinical improvement for more than 18 months. nasal mask intermittent positive pressure venSuccessful tilation (NIPPV) has recently been reported in respira-

tory failure due to neuromuscular diseases by several authors.!" We wish to report two cases of Duchennes muscular dystrophy (DMD) treated with NIPPV during the hours of sleep. In both cases, there has been sustained clinical and physiologic improvement for more than 18 months. Both patients reported an increased sense of well being, absence of previously reported daytime hypersomnolence, and less daytime dyspnea. The effectiveness of this technique is graphically demonstrated. CASE REPORTS CASE

1

A 29-year-old white man had DMD diagnosed at age five years. At age 28 years, he complained of dyspnea at night, daytime hypersomnolenee, headaches, and palpitations. On physical examination, he was unable to maintain a sitting position without support. There was severe generalized muscle wasting with contractures of the legs. There was deformity of the chest with pectus carinatum and kyphoscoliosis. The pulse was 140 beats per minute, respirations, 30 per minute, and shallow. The FVC was 460 ml or 10 percent predicted. An arterial blood gas (ABG) analysis awake on room air yielded the following results: pH, 7.37; PaC02 , 60 mm Hg, and PaD!, 55 mm Hg. He was admitted to the hospital and placed on NIPPV. Two months following discharge, using NIPPV at night at home, he was much improved. He was less short of breath during the day with no daytime hypersomnolence. The FVC was 520 ml. An ABC analysis awake on room air yielded the following results: pH, 7.36; PaC02, 50; Pa02, 69. Figure 1 shows results of continuous transcutaneous recordings of Pco, and P0 2 on room air and subsequently on NIPPV at a rate of 15 breaths per minute, and Vr; 400 ml on room air. The tcf'co, fell from a mean of72.3 mm Hg to 43.2 mm Hg. The tcf'o, increased from a mean of37.6 mm Hg to 61.8 mm Hg. Ear oximetry findings ranged from a low of71 percent O 2 saturation without support to a high of96 *Fom the Monmouth Medical Center, Long Branch, N]. Presented before the New Jersey Chapter, American Thoracic Society, June 4, 1986. Reprint requests: Dr. Segall, 255 Third Avenue, Long Branch, N] 07740 Noninvasive Nasal Mask-assisted Ventilation (DaVid Segall)

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percent saturation on NIPP~ Marked improvement in chest excursion and breath sounds were observed on NIPPV. Clinical improvement has been maintained for more than 18 months. CASE

developed respiratory failure at age 14 years, when an ABC analysis yielded the following results: pH, 7.35; PaCO., 49 mm Hg; PaO., 49 mm Hg. He was treated with nasal oxygen at lUmin, but subsequently developed hypercapnia. He was admitted for observation. On room air awake, ABC values were as follows:pH, 7.36; Paeot , 60 mm Hg, PaO., 55 mm Hg. During sleep, unassisted on room air, the

2

A IS-year-oldwhite man had DMD diagnosed at age two years. He

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CHEST / 93 / 6 / JUNE. 1988

1299

tcf'o, fell from 63 mm Hg to 43 mm Hg. On NIPPV with 24 percent inspired oxygen, there was correction of hypoxemia without hypercapnia. Subsequently, supplementary oxygen was not required. Twomonths after discharge using NIPPV at night, he reported an increased sense of well being with no headaches, an improved daytime performance, including schoolwork, and absence of previously reported daytime hypersomnolence. A repeat study after five months showed sustained improvement. An ABGanalysis on room air awake without assistance yielded values as follow: pH, 7.39; PaC02, 48 mm Hg; Pa02, 76 mm Hg. Spirometry showed VC of 460 ml, VE, 4.75 Umin; VT, 153 ml: respirations, 31 per minute. During unassisted sleep, Pa02 fell from 76 mm Hg to a low of 53 mm Hg. On NIPPV on room air Pa02 increased to a maximum of97 mm Hg (range 97 to 73 mm Hg). The PaC02 was now normal during unassisted sleep and fell slightly on NIPPV (Fig 2). Improvement has been maintained for more than 18 months using NIPPVon room air for sleep at home. DISCUSSION

These two case studies confirm the effectiveness of noninvasive nasal mask ventilation in the treatment of respiratory failure of DMD. The nasal mask used is available for treatment of obstructive sleep apnea and its application has been well described. 6-9 Long-term survival using prolonged assisted ventilation, after the onset of respiratory failure in DMD, has been well documented by multiple authors'? and needs to be emphasized. In 1973, Aberion et al" reported three cases. Curran" presented nine patients treated with a negative pressure tank ventilator or cuirass. The average treatment was two years with survival up to four years to the age of 28 years. Alexander et al" reported ten cases with survival from two to 7.5 yrs (three died). Their methods included volume ventilation by tracheostomy rocking bed, plastic wrap assistance, and chest-abdomen cuirass. Rideau et aliiin France and Bach et al" have reported extensively, the latter including up to 11 years' survival with 19 patients living in the community and four "earning a living." Their methods included mouth intermittent positive pressure ventilation (MIPPV) employing a lip seal at night. In France, NIPPV has been employed in paralytic respiratory failure'" in 30 patients providing assistance during sleep and rest. In a preliminary report," the use of NIPPV has been described to permit. dental surgery in a patient with DMD who was dependent on MIPPV. In another case, transient respiratory failure complicating multiple sclerosis was managed with NIPPV avoiding endotracheal intubation. Prolonged use of NIPPV in neuromuscular respiratory failure was reported in three cases. In another study," five patients with chronic respiratory failure have been treated with NIPPV with sustained clinical and physiologic improvement. These included two cases of DMD. The most comprehensive study appears to be that of Ellis et al" employing polysomnography in a sleep laboratory. They compared negative pressure ventilation with NIPPV in five patients, including one with DMD and four with other neuromuscular diseases. All patients had severe hypoxemia during control sleep, being most severe during REM-like sleep. These episodes were due to upper airways obstructive apneas with complete cessation of oronasal air Bowand were not prevented by negative pressure ventilation. By contrast, 1300

NIPPV was effective in maintaining oxygenation in all patients during both non-REM and REM sleep and was associated with restoration of normal sleep patterns. Four patients have been treated at home with NIPPV for periods up to 12 months. CONCLUSION

The present cases confirm previous observations of the value of noninvasive nasal mask ventilation in respiratory failure ofDMD. They demonstrate correction of hypoxemia by improvement in ventilation without the use of supplementary oxygen. The improvement during the daytime when NIPPV is not in use, is probably due to the prevention of microatelectasis during sleep, by increasing nighttime tidal volume. The improvement in daytime hypersomnolence is clearly due to improvement in the quality of sleep as demonstrated by Ellis et ale 5 It is thought that this method should be more widely applied in respiratory failure due to muscular dystrophy and other neuromuscular diseases. ACKNOWLEDGMENT: We wish to acknowledge the assistance of Donna Lempka, RRT and Lou Saporito RRT in preparation of this study. REFERENCES

2 3 4 5 6 7 8 9 10 11 12 13 14

Delaubier A. Traitement de l'insuffisance repiratoire chronique dans les dystrophies musculaires. In: Memoires de certificat d'etudes superieures de reeducation et readaptation fonctionnelles. Paris: University R Descartes, 1984:1-124 Rideau Y. Management of the wheelchair muscular dystrophy patient: prevention of death (abstract). 4th International Congress on Neuromuscular Diseases, Los Angeles, 1986 Bach J, Alba A, Mosher R, Delaubier A. Intermittent positive pressure ventilation via nasal access in the mangement of respiratory insufficiency. Chest 1987; 92:168-70 Kerby G, Mayer L, Pingleton S. Nocturnal positive pressure ventilation via nasal mask. Am Rev Respir Dis 1987; 135:738-40 Ellis E, Bye ~ Bruderer J, Sullivan C. Treatment of respiratory failure during sleep in patients with neuromuscular disease. Am Rev Respir Dis 1987; 135:148-52 Sullivan C, Issa FG, Berthon-Jones M. Reversal of obstructive sleep apnea by continuous positive airways pressure applied through the nares. Lancet 1981; 1:862-65 Rapoport DM, Sorkin B, Garay SM, Goldring RM. Reversal of the pickwickian syndrome by long term use of nocturnal nasal airway pressure. N Eng} J Med 1982; 307:931-33 Sanders M, Moore SE, Eveslage J. CPAP via nasal mask: a treatment for occlusive sleep apnea. Chest 1983; 1:144-45 Rapoport DM, Garay SM, Goldring RM. Nasal CPAP in obstructive sleep apnea mechanism of action. Bull Europ Physiopath Respir 1983; 19:616-20 Aberion G, Alba A, Lee MHM, Solomon M. Pulmonary care of Duchennes type of muscular dystrophy. NY State J Med 1973; 15:1206-07 Curran FJ. Night ventilation by body respirators in chronic respiratory failure due to late stage Duchennes muscular dystrophy. Arch Phys Med Rehabill981; 62:270-74 Rideau Y, Gatin G, Bach J, Gimes G. Prolongation of life in Duchennes muscular dystrophy. Acta Neuro11983; 5:118-24 Bach J, O'Brien J, Krotenberg R, Alba A. Management of end stage of respiratory failure in Duchennes muscular dystrophy. Muscle & Nerve 1987; 10:177-82 Alexander M, Johnson FW, Petty J, Stauch D. Mechanical ventilation of patients with late stage Duchennes muscular dystrophy; management in the home. Arch Phys Med Rehabil 1879 60:289-92 Noninvasive Nasal Mask-assisted Ventilation (David Segall)