65 Tüberküloz ve Toraks Dergisi 2005; 53(1): 65-68
Pulmonary hypertension and acute pulmonary edema in a 23-year-old male with a history of an upper respiratory tract infection
A. Füsun ÜLGER, Elif ŞEN, Akın KAYA, Turan ACICAN, Öznur AKKOCA, Gülseren KARABIYIKOĞLU Ankara Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, Ankara.
ÖZET
Yirmiüç yaşında bir erkekte üst solunum yolu infeksiyonu sonrası gelişen pulmoner hipertansiyon ve akut akciğer ödemi
Üst hava yolu obstrüksiyonu patofizyolojisi oldukça karmaşıktır. Akut solunum yetmezliğine neden olabilecek nedenler ara- sında akut epiglottit ya da boğmaca gibi infeksiyonlar, dil kökü, larenks ve hipofarenkste obstrüksiyona neden olan tümör- ler, aspire edilen katı ve sıvı gıdalar, obezite ve anatomik varyasyonlar sayılabilir. Ciddi karbondioksit retansiyonu ve apne acil endotrakeal entübasyonu gerektirebilmektedir. Makalede, 23 yaşında, üst solunum yolu varyasyonu nedeniyle, üst so- lunum yolu infeksiyonu, pulmoner ödem ve pulmoner hipertansiyon ile komplike olmuş bir olgu sunulmaktadır. Olgu na- dir görülen ve ağır bir tablo ile başvurmuş, etyolojik olarak ayrıntılı bir şekilde değerlendirilmiş ve yaşama geri dönmüştür.
Anahtar Kelimeler: Üst solunum yolu obstrüksiyonu, pulmoner ödem, pulmoner hipertansiyon.
SUMMARY
Pulmonary hypertension and acute pulmonary edema in a 23-year-old male with a history of an upper respiratory tract infection
Ulger AF, Sen E, Kaya A, Acican T, Akkoca O, Karabiyikoglu G
Department of Chest Diseases, Faculty of Medicine, Ankara University, Ankara, Turkey.
The pathophysiology of upper-airway obstruction (UAO) is complex. Possible causes of UAO that may lead to acute respi- ratory failure, are as follows: infections like acute epiglottitis and croup, obstructing tumors in the base of the tongue, lar- ynx or hypopharynx, aspirated food or liquid contents, obesity and anatomical variations. Management changes accord- ing to the pathogenesis of the disorder. In patients with severe carbon dioxide retention or apnea, emergency endotracheal intubation must be carried out. Hereby, we describe a 23-year-old patient with susceptible upper-airway anatomy and UAO occured following an upper respiratory infection and complicated with pulmonary hypertension and pulmonary edema. Our patient seems to be one of the complicated UAO cases, with an unusual but critical clinical presentation, eval- uated in a wide spectrum and nicely returned to life.
Key Words: Upper-airway obstruction, pulmonary edema, pulmonary hypertension.
Yazışma Adresi (Address for Correspondence):
Dr. A. Füsun ÜLGER, Ankara Üniversitesi Tıp Fakültesi, Göğüs Hastalıkları Anabilim Dalı, 06100, Dikimevi, ANKARA - TURKEY
e-mail: [email protected]
The pathophysiology of upper-airway obstructi- on (UAO) is complex and still under active in- vestigation. Possible causes of UAO that may le- ad to acute respiratory failure, are as follows: in- fections like acute epiglottitis and croup, obst- ructing tumors in the base of the tongue, larynx or hypopharynx, aspirated food or liquid con- tents, obesity and anatomical variations. Mana- gement changes according to the pathogenesis of the disorder. In patients with severe carbon di- oxide retention or apnea, emergency endotrac- heal intubation must be carried out (1).
With sleep onset, the wakefulness stimulus is lost and the negative pressure reflex is diminis- hed. Thus, within a short time after sleep onset, upper airway muscle activity generally decre- ases and upper-airway resistance increases, even in normal subjects. With sustained sleep, the activity of the genioglossus may return to waking levels, but the activity of the palate muscles often remains below that during wake- fulness. Thus, susceptible upper-airway ana- tomy and a loss of upper-airway muscle activity are the key elements UAO (2).
The major site of upper-airway narrowing in nor- mal subjects appears to be in the retropalatal area (3). Especially in predisposed, anatomi- cally variant individuals, sleep disorders should gain importance when complicated with other circumstances.
Hereby, we describe a patient with susceptible upper-airway anatomy and UAO occured follo- wing an upper respiratory infection and compli- cated with pulmonary hypertension and pulmo- nary edema.
CASE REPORT
A 23-year-old male presented with slowly prog- ressive dyspnea following an upper respiratory tract infection, cyanosis, and swelling of face and legs for last two months, on December 2002. His abdominal ultrasonographic examination, on No- vember 2002, has revealed dilatation of inferior vena cava and hepatic veins with right pleural ef- fusion and invasive hemodynamic study has shown pulmonary artery dilatation with systolic pulmonary arterial pressure (PAP) measured as
65 mmHg. His clinical course quickly worsened within hours and he was entubated and his follow- up was performed in intensive care unit.
His past medical history was perfect except his mental retardation. Physical examination reve- aled bilateral decreased breath sounds at both bases, fine crackles up to both apices on auscul- tation, S3 gallop rhythm, hepatomegaly, pretibi- al edema and jugular venous distension. His blo- od gas measurements necessitating entubation revealed the following: pH: 7.14, PaCO2: 158.1 mmHg, PaO2: 7.4 mmHg, and oxygen saturati- on: 2.9%. Complete blood count, blood che- mistry and urin analysis were normal. The chest radiography revealed changes that were consis- tent with cardiomegaly with enlarged pulmonary conus, bilateral pleural effusion and pulmonary edema (Figure 1). Electrocardiographic exami- nation showed incomplete right bundle branch block. Congestive heart failure was diagnosed and diuretics with ACE inhibitor therapy was be- gun. Clinical response were quiet perfect and he was extubated on the second day; and his high resolution computed tomography (CT) of thorax on day four was compatible with cardiogenic pulmonary edema. On echocardiography, systo- lic PAP was 85 mmHg.
Nocturnal desaturations and apnea following sleep were observed during follow-up and bile-
Pulmonary hypertension and acute pulmonary edema in a 23-year-old male with a history of an upper respiratory tract infection
Tüberküloz ve Toraks Dergisi 2005; 53(1): 65-68 66
Figure 1. Chest X-ray of the patient on admission which reveals changes that were consistent with car- diomegaly with enlarged pulmonary conus, bilateral pleural effusion and pulmonary edema.
Ülger AF, Şen E, Kaya A, Acıcan T, Akkoca Ö, Karabıyıkoğlu G.
Tüberküloz ve Toraks Dergisi 2005; 53(1): 65-68 vel positive airway pressure (BiPAP) during sle-
ep time was commenced. His blood gas measu- rements on the seventh day of BiPAP usage re- vealed the following: pH: 7.39, PaCO2: 51.1 mmHg, PaO2: 78 mmHg, and oxygen saturati- on: 92%. Clinical picture was under control; but aetiological investigations for rapidly progressi- ve pulmonary hypertension and sleep disorder were performed.
Cranial MRI and neck CT were normal. No ne- urological abnormality causing sleep disorder was found. Psychometric evaluation was perfor- med and high cortical functional evaluation re- port informed that his IQ was 35-40. Ear-nose- throat examination revealed narrowed left nasal cavity with septum deviation. Distance between nasopharynx and soft palate was narrow and occluding during snorring. Epiglottis was omega shaped and collapsing with deep inspiration.
Surgical indication for septum deviation and vo- lume reduction of soft palate was present; but his mental retardation was a contraindication.
Polisomnographic study showed nocturnal de- saturation (mean saturation: 70%), five obstruc- tive and one central apnea and 19 hipopnea.
Mean apnea duration was 53 seconds (84 sec, max). Apnea-hipopnea index was 3.6 and BiPAP was suggested during sleep.
On January 2003, his systolic PAP was 40 mmHg with minimally enlarged right heart. He was discharged to be on follow-up. Echocardi- ography on March 2003 was a victory: Normal cardiac dimensions with systolic PAP measured as 20 mmHg. He is still well with normal blood gas analysis and normal radiology (Figure 2).
DISCUSSION
The patency of the upper-airway depends on extraluminal tissue/gravitation factors, anatomi- cal factors, the amount of negative intraluminal pressure, as well as amount of upper-airway muscle activity (4).
In polysomnographic study, mixed apneas and hypopneas were determined in our patient. Mi- xed apneas are those in which the initial portion of the apnea is central and remaining portion is obstructive. The postapnea ventilatory period
may have consequences if the patient returns to sleep quickly. If the PaCO2 falls below a point called the “apneic threshold”, then respiratory drive ceases, resulting in central apnea. This is thought to be the origin of the central portion of mixed apnea. Periods of reduced tidal volume or airflow during hypopneas may also be associ- ated with desaturations or sleep disturbance (2).
Although those could be due to a fall in central drive or partial airway obstruction, a seperation is not always possible. Stabilization of the upper airway by nasal continuous positive airway pressure (CPAP) or BiPAP usually abolishes both components of mixed apnea. BiPAP quickly improved the clinical picture and stabilized the laboratory parameters in our young patient.
Like in patients with bony abnormality, obstruc- tive sleep apnea, long soft palate, a large poste- riorly placed tongue, increased fat deposition, or tissue edema; a possible obstruction to airflow occured in our patient during sleep at one or se- veral locations in upper-airway. To our suggesti- on, his story began with an upper airway infec- tion with tissue edema. In addition, he had an anatomical variation with a narrow distance bet- ween nasopharynx and soft palate occluding du- ring snorring and an omega-shaped epiglottis collapsing with deep inspiration. So, he had had an increased supraglottic resistance and, unlike normal subjects, required a positive intraluminal pressure to prevent airway closure during sleep.
67
Figure 2. Patient’s control chest X-ray was normal.
His mental retardation which was a contraindi- cation for septum deviation and soft palate volu- me reduction surgery necessitated BiPAP admi- nistration.
Pulmonary arterial blood pressure rises during apneas and then falls when the oxygen saturati- on returns to normal after apnea. The main ca- use is hypoxic vasoconstriction. In addition, an increase in systemic blood pressure is also asso- ciated with each obstructive apnea/hypopnea (2). Congestive heart failure and increased PAP was present in our case, which was thought to occur due to his sleep disorder caused by his anatomical variation.
One of the probable explanations of our pati- ent’s clinical picture was pulmonary edema which may complicate UAO in both children and adults. The mechanism for the formation of pul- monary edema was elucidated in analysis of three cases, showing a hydrostatic mechanism for edema fluid formation (5). In another study, the cause was attributed to pulmonary and he- modynamic changes resulting from high negati- ve intrathoracic pressures during obstructed res- piration (6). The incidence and the reasons of pulmonary edema development in only certain patients are unclear (5,6).
In conclusion, our patient seems to be one of the complicated upper-airway obstruction cases, with an unusual but critical clinical presentation, evaluated in a wide spectrum and nicely retur- ned to life.
REFERENCES
1. Matthay MA, Atabai K. Acute hypercapnic respiratory failure: neuromuscular and obstructive diseases. In: Ge- orge RB, Light RW, Matthay MA, Matthay RA (eds).
Chest Medicine: Essentials of Pulmonary and Critical Ca- re Medicine. Philadelphia: Lippincott Williams and Wil- kins, 2000: 561-75.
2. Berry RB. Sleep-related breathing disorders. In: George RB, Light RW, Matthay MA, Matthay RA (eds). Chest Me- dicine: Essentials of Pulmonary and Critical Care Medici- ne. Philadelphia: Lippincott Williams and Wilkins, 2000:
478-502.
3. Schwab RJ. Upper airway imaging. Clin Chest Med 1998; 19: 33-54.
4. Van de Graaff WB. Thoracic influence on upper airway patency. J Appl Physiol 1988; 65: 2124-31.
5. Kallet RH, Daniel BM, Gropper M, Matthay MA. Acute pul- monary edema following upper airway obstruction: case reports and brief review. Respir Care 1998; 43: 476-80.
6. Tami TA, Chu F, Wildes TO, Kaplan M. Pulmonary ede- ma and acute upper airway obstruction. Laryngoscope 1986; 96: 506-9.
Pulmonary hypertension and acute pulmonary edema in a 23-year-old male with a history of an upper respiratory tract infection
68 Tüberküloz ve Toraks Dergisi 2005; 53(1): 65-68
