Pulmonary Varix Regression
IRENE HSU, GEORGE A.
MD KELSER,
MD
PAUL C. ADKINS, MD MICHAEL M. SHEFFERMAN. Washington,
MD
D. C.
After Mitral Valve Replacement
Pulmonary varix is a rare finding; only 35 documented cases have been reported. The first case was described in 1843 as an incidental postmortem finding. The first clinical diagnosis was not made until 1951. In more than half of the 35 cases, the varix was present in the absence of congenital and acquired heart disease. Six patients have had concomitant mitral rheumatic heart disease. This communication describes the second patient with rheumatic mitral regurgitation in whom the pulmonary varix became radiographically invisible after prosthetic mitral valve replacement.
Varices of pulmonary veins are rare. Puchelt’ is credited with being the first to describe it in 1843 as an incidental postmortem finding in a newborn who died from intestinal hemorrhage and had multiple varices in other organs as well. Subsequently five additional cases were reported,‘-’ all diagnosed at postmortem examination. The first clinical diagnosis was not made until 1951 when Mouqin et al.8 established the diagnosis of a pulmonary varix in a patient by angiography. Eight years elapsed before a second case was reportedg; as in Mouqin’s case, the diagnosis was made by pulmonary angiography. To date, only 35 documented cases have been published,lmlg the majority since 1959. This communication describes a patient with concomitant rheumatic heart disease whose pulmonary varix is no longer visible by X-ray examination 2 years after successful mitral valve replacement with a Starr-Edwards prosthesis. To our knowledge, only one similar case has previously been reported. lo Case Report
From the Department of Medicine and Surgery, The George Washington University Medical Center, Washington, (3. C. Manuscript accepted January 8, 1975. Address for reprints: Irene Hsu, MD, The George Washington University Medical Center, 2150 Pennsylvania Avenue, N. W.. Washington, D. C. 20037.
928
May 1976
The American
The patient is a 44 year old black housewife admitted to the George Washington University Hospital on February 24, 1966 with infectious endocarditis due to Streptococcus viridans. Asymptomatic rheumatic heart disease with mitral insufficiency had been present for many years. The endocarditis responded well to treatment with penicillin and streptomycin and the patient was discharged free of symptoms in 6 weeks. Follow-up chest X-ray film of March 29, 1967. (Fig. 1) revealed a rounded right paracardiac density that was not present on Februarv 5, 1966. Tomography (Fig. 21 localized it at 9.5 to 10 cm in the anteroposterior projection. Its configuration suggested a vascular anomaly. There was no change in the patient’s clinical status and no personal or family history of hemoptysis or other bleeding tendency. Right heart catheterization (Table I) and forward pulmonary cineangiography with filming continued into the levophase identified the density as a right inferior pulmonary varix. In the ensuing 4 years heart and varix size increased (Fig. 3), but the patient remained free of symptoms aside from episodic nocturnal cough. Hemodynamic studies were repeated on June 18, 1971 (Table I). In view of significant mitral regurgitation with increasing pulmonary arterial pressure, and particularly because of progressive enlargement of the pulmonary varix, the patient underwent prosthetic replacement of mitral valve on July 13, 1971.
Journal of CARDIOLOGY
Volume
37
PULMONARY
VARIX-HSU
FIGURE 1. Chest roentgenograms. Left, film of March 29, 1967 demonstrates enlarged cardiac silhouette and a rounded density lower lung field adjacent to the cardiac border. Right, earlier film of February 25, 1966 reveals no abnormal findings.
a large varix of the right inferior pulmoAt operation, nary vein was visualized. Its posterior location precluded adequate photographic documentation. Reoperation was required on March 2, 1972 for correction of a paravalvular leak with resultant severe pulmonary hypertension (Table I) and congestive heart failure. A defective Starr-Edwards no. 6300 stellite ball valve prosthesis was replaced with a no. 6120 Silastic@ ball valve. Postoperatively, symptoms and signs of congestive heart failure disappeared and heart and varix size diminished. At present the varix can no longer be identified by chest X-ray film (Fig. 4), although it is still discernible by tomography. Discussion
A pulmonary varix can be defined as a localized enlargement of a pulmonary vein that enters normally into the left atrium. It is a rare entity of uncertain origin. Developmental aberrations during the primitive splanchnic capillary stage are considered to be a likely cause,12 particularly when the varix is seen in asymptomatic healthy young peop1e.l’ In the symptomatic group with pulmonary venous hypertension, varicosities have been attributed to long-standing pulmonary venous hypertension.1°J2J3 Since the majority of patients with chronic pulmonary venous hypertension do not have associated pulmonary varices, it seems likely that an additional local factor may exist. In our patient its appearance subsequent to infectious endocarditis raises a possibility that infection of the venous wall played a causative role; however, hemodynamic worsening of mitral insufficiency appears more plausible. Clinical
ET
AL.
in the right
differentiated from other “coin lesions” as well as from pulmonary arteriovenous fistula and anomalous pulmonary venous drainage.‘s Unnecessary thoracotomy may be avoided if pulmonary varix is included in the differential diagnosis. 1g,20 Definitive diagnosis is established by pulmonary angiography. The most common site for a varix is the right upper pulmonary lobe; the next, the left upper lobe and lingula.16 Varices are usually single, but occasionally may be multiple as illustrated by one patient who had five in the left upper pulmonary lobe.7 Pulmonary varices usually do not cause symptoms and are generally considered benign.‘l,ls Yet pulmonary varix caused sudden death in two patients as a result of spontaneous rupture of the varix into the pleural cavity4J5 and it contributed to death in two other
Features
Pulmonary varix usually comes to attention because of abnormal chest X-ray findings and must be
FIGURE 2. Tomogram at 9.5 cm in anteroposterior highly suggestive of a vascular density.
May
1976
The American
Journal
of CARDIOLOGY
Volume
projection
37
is
929
PULMONARY VARIX-HSU
TABLE
ET AL
I
Cardiac Catheterization
Findings October
Pressures (mm Hg) Right atrium Mean c wave ” wave Right ventricle Pulmonary artery Pulmonary “capillary” Mean I/ wave Left atrium Mean c wave II wave Left ventrrcle Aorta Left brachial artery 0, saturation Nitrous oxide from pulmonary artery Fiberoptic catheter exploration of RA and SVC lndocyanine green curves Pulmonary cineangiogram
1967
June 1971
2
3 62/i
32&/O 30-35/8-l
2 (25)
FIGURE
18-22 10 45 11518 115182
i&i
3. Chest roentgenograms
WC
May 1976
The American
1 I’;,; 6 115180
Negative for shunts Varix right Inferior pulmonary vein Marked mitral regurgitation
RA = right atrium;
22 36 24 lOOl22 100/55 (80)
3% (normal) Negative
Marked paravalvular regurgitation
= superior vena cava
(posteroanterior
and lateral) of June 13. 1971 reveal an increase
Journal of CARDIOLOGY
Volume
in heart and varix size since 1967
quired heart disease. Six patients had concomitant mitral rheumatic heart disease. In one, the size of the varix decreased after successful treatment of congestive heart failure.” TwogJo of the six patients underwent surgery for mitral valve disease. Mitral commissurotomy was performed in one patient9 whose varix had not enlarged in 5 years; 4 years postoperatively the radiographic appearance of the varix was unchanged. Prosthetic mitral valve replacement was performed in the other,lO whose varix had progres-
cases. In one” of the latter two, it communicated with a bronchus as a result of erosion into the varix by an adjacent tuberculous process, and recurrent hemoptysis led to death. In the other, the varix contained thrombi thought to be the source of cerebral emboli that resulted in hemiplegia and death.” In all four patients the diagnosis of pulmonary varix was not made until postmortem examination. In more than half of the reported cases, the varix was present in the absence of either congenital or ac-
930
(46)
1972
14-18 20-25
LV cineangiogram
LV = left ventricle;
62/32
February
37
PULMONARY VARIX-HSU
FIGURE
4. Chest roentgenograms
(posteroanterior
and lateral) of May 20. 1974. The pulmonary
sively enlarged for 7 years, 2 years postoperatively the varix was no longer visible radiographically.
ET AL.
varix is no longer visible.
pulmonary varix after mitral valve replacement in the patient described by Hipona and JamshidiiO and in our patient supports the recommendation of operative treatment in this group of patients with pulmonary varix.
Treatment The limited clinical experience with pulmonary varix precludes definitive therapeutic recommendation, but treatment does not appear to be indicated in asymptomatic young healthy adults once the diagnosis is firmly established.‘l Serial observations, including chest roentgenograms, to determine stability of the lesion may be prudent in this group. Nevertheless, it bears emphasis that onels of the two patients who died suddenly from spontaneous rupture of the varix into the pleural cavity was a 30 year old woman without cardiac or pulmonary disease whose varix size was unchanged radiographically for 12 years. In symptomatic persons with pulmonary venous hypertension due to mitral valve disease, replacement of the diseased valve by prosthesis should be given consideration, especially if the varix has progressively enlarged. Roentgenographic disappearance of the
Addendum Since completion of this manuscript, a brief case report by Perrott and Shinzl has appeared, describing a 33 year old woman with rheumatic mitral insufficiency and a pulmonary varix of the right inferior pulmonary vein. The varix was visualized at the time of “valvular surgery,” but the subsequent course was not stated. Acknowledgment our appreciation to Drs. Michael Paszek and Wilkenfeld for their assistance in the preparation of
We express
Jack
the manuscript typing.
and to Mrs.
Marlyn
Baran
for her patient
References 1. Puchelt: quoted by Gimes R, Horvath F: Uber die Varikositat de Pulmonaivene. Fortschr Rontgenstr 89:545-548. 1958 eines Lungenvarix. Verhandlungen 2. Hedinger E: Demonstration der Deutschen Gesellschaft fur Pathologie 11:303-308, 1907
7.
Hauwerk D: Lungenvarix und Haemoptose. Munchener Med Wochenschrift 70: 1084-1086, 1923 4. Klinck GH Jr, Hunt HD: Pulmonary varix with spontaneous rupture and death: report of a case. Arch Pathol 15:227-237, 1933 5. Neiman BH: Varix of pulmonary vein. Am J Roentgen01 32: 608-612, 1934 P: Phlebektasie im Lungenparenchym (tin Beitrag zu 6. Jacchia
3.
8.
9.
IO.
May 1976
den isolierten Rundschatten in der Lunge). Acta Radio1 17:7487, 1936 Case records of the Massachusetts General Hospital, N Engl J Med 245~575-581, 1951 Mouquin M, Hebrard H, Damasio R, et at: Varice du poumon diagnostiqub par I’ angiocardiographie. Bull Mem Sot Med Hop Paris 67:1091-1094, 1951 Gottesman L, Weinstein A: Varicosities of the pulmonary veins: Case report and survey of the literature. Dis Chest 35:322-327, 1959 Hipona FA, Jamshidi A: Observation on the natural history of
The American
Journal
of CARDIOLOGY
Volume
37
931
PULMONARY
varicosity 11. 12. 13. 14.
15. 16.
932
VARIX-HSU
ET AL
of pulmonary
veins. Circulation
35:471-475.
927-935, 197 1 Meszaros W: Lung changes in left heart failure. Circulation 47: 859-871, 1973 18. Papamichael E, lkkas D, Alkalais K, et al: Pulmonary varicosity associated with other congenital abnormalities. Chest 62: 107-109, 1972 19. Davia JE, Golden MS, Price HL, et al: Pulmonary varix. A diagnostic pitfall. Circulation 49:lOi l-1012, 1974 20. Bryk D, Levin EJ: Pulmonary varicosity. Radiology 85:835-837, 1965 21. Perrott WW, Shin MS: Pulmonary varix. J Thorac Cardiovasc Surg 68:318-320, 1974
1967
17.
Nelson WP, Hall RJ, Garcia E: Varicosi& of the pulmonary veins simulating arteriovenous fistula. JAMA 195:13-17, 1966 Poller S, Wholey MH: Pulmonary varix: evaluation by selective pulmonary angiography. Radiology 86: 1078-l 081, 1966 Bryk D: Dilated right pulmonary veins in mitral insufficiency. Chest 58:24-27. 1970 Viamonte M, Le Page JR: Pitfalls in the radiologic evaluation of mediastinal abnormalities. Radio1 Clin North Am 6:451-465, 1968 Perret L, Fortelius P: Ruptured aneurysm of the pulmonary vein. Acta Tuberc Stand 41:53-54. 1961 Bartram C, Strickland B: Pulmonary varices. Br J Radio1 44:
May 1976
The American
Journal
of CARDIOLOGY
Volume
37
IRENE HSU, GEORGE A.
MD KELSER,
MD
PAUL C. ADKINS, MD MICHAEL M. SHEFFERMAN. Washington,
MD
D. C.
After Mitral Valve Replacement
Pulmonary varix is a rare finding; only 35 documented cases have been reported. The first case was described in 1843 as an incidental postmortem finding. The first clinical diagnosis was not made until 1951. In more than half of the 35 cases, the varix was present in the absence of congenital and acquired heart disease. Six patients have had concomitant mitral rheumatic heart disease. This communication describes the second patient with rheumatic mitral regurgitation in whom the pulmonary varix became radiographically invisible after prosthetic mitral valve replacement.
Varices of pulmonary veins are rare. Puchelt’ is credited with being the first to describe it in 1843 as an incidental postmortem finding in a newborn who died from intestinal hemorrhage and had multiple varices in other organs as well. Subsequently five additional cases were reported,‘-’ all diagnosed at postmortem examination. The first clinical diagnosis was not made until 1951 when Mouqin et al.8 established the diagnosis of a pulmonary varix in a patient by angiography. Eight years elapsed before a second case was reportedg; as in Mouqin’s case, the diagnosis was made by pulmonary angiography. To date, only 35 documented cases have been published,lmlg the majority since 1959. This communication describes a patient with concomitant rheumatic heart disease whose pulmonary varix is no longer visible by X-ray examination 2 years after successful mitral valve replacement with a Starr-Edwards prosthesis. To our knowledge, only one similar case has previously been reported. lo Case Report
From the Department of Medicine and Surgery, The George Washington University Medical Center, Washington, (3. C. Manuscript accepted January 8, 1975. Address for reprints: Irene Hsu, MD, The George Washington University Medical Center, 2150 Pennsylvania Avenue, N. W.. Washington, D. C. 20037.
928
May 1976
The American
The patient is a 44 year old black housewife admitted to the George Washington University Hospital on February 24, 1966 with infectious endocarditis due to Streptococcus viridans. Asymptomatic rheumatic heart disease with mitral insufficiency had been present for many years. The endocarditis responded well to treatment with penicillin and streptomycin and the patient was discharged free of symptoms in 6 weeks. Follow-up chest X-ray film of March 29, 1967. (Fig. 1) revealed a rounded right paracardiac density that was not present on Februarv 5, 1966. Tomography (Fig. 21 localized it at 9.5 to 10 cm in the anteroposterior projection. Its configuration suggested a vascular anomaly. There was no change in the patient’s clinical status and no personal or family history of hemoptysis or other bleeding tendency. Right heart catheterization (Table I) and forward pulmonary cineangiography with filming continued into the levophase identified the density as a right inferior pulmonary varix. In the ensuing 4 years heart and varix size increased (Fig. 3), but the patient remained free of symptoms aside from episodic nocturnal cough. Hemodynamic studies were repeated on June 18, 1971 (Table I). In view of significant mitral regurgitation with increasing pulmonary arterial pressure, and particularly because of progressive enlargement of the pulmonary varix, the patient underwent prosthetic replacement of mitral valve on July 13, 1971.
Journal of CARDIOLOGY
Volume
37
PULMONARY
VARIX-HSU
FIGURE 1. Chest roentgenograms. Left, film of March 29, 1967 demonstrates enlarged cardiac silhouette and a rounded density lower lung field adjacent to the cardiac border. Right, earlier film of February 25, 1966 reveals no abnormal findings.
a large varix of the right inferior pulmoAt operation, nary vein was visualized. Its posterior location precluded adequate photographic documentation. Reoperation was required on March 2, 1972 for correction of a paravalvular leak with resultant severe pulmonary hypertension (Table I) and congestive heart failure. A defective Starr-Edwards no. 6300 stellite ball valve prosthesis was replaced with a no. 6120 Silastic@ ball valve. Postoperatively, symptoms and signs of congestive heart failure disappeared and heart and varix size diminished. At present the varix can no longer be identified by chest X-ray film (Fig. 4), although it is still discernible by tomography. Discussion
A pulmonary varix can be defined as a localized enlargement of a pulmonary vein that enters normally into the left atrium. It is a rare entity of uncertain origin. Developmental aberrations during the primitive splanchnic capillary stage are considered to be a likely cause,12 particularly when the varix is seen in asymptomatic healthy young peop1e.l’ In the symptomatic group with pulmonary venous hypertension, varicosities have been attributed to long-standing pulmonary venous hypertension.1°J2J3 Since the majority of patients with chronic pulmonary venous hypertension do not have associated pulmonary varices, it seems likely that an additional local factor may exist. In our patient its appearance subsequent to infectious endocarditis raises a possibility that infection of the venous wall played a causative role; however, hemodynamic worsening of mitral insufficiency appears more plausible. Clinical
ET
AL.
in the right
differentiated from other “coin lesions” as well as from pulmonary arteriovenous fistula and anomalous pulmonary venous drainage.‘s Unnecessary thoracotomy may be avoided if pulmonary varix is included in the differential diagnosis. 1g,20 Definitive diagnosis is established by pulmonary angiography. The most common site for a varix is the right upper pulmonary lobe; the next, the left upper lobe and lingula.16 Varices are usually single, but occasionally may be multiple as illustrated by one patient who had five in the left upper pulmonary lobe.7 Pulmonary varices usually do not cause symptoms and are generally considered benign.‘l,ls Yet pulmonary varix caused sudden death in two patients as a result of spontaneous rupture of the varix into the pleural cavity4J5 and it contributed to death in two other
Features
Pulmonary varix usually comes to attention because of abnormal chest X-ray findings and must be
FIGURE 2. Tomogram at 9.5 cm in anteroposterior highly suggestive of a vascular density.
May
1976
The American
Journal
of CARDIOLOGY
Volume
projection
37
is
929
PULMONARY VARIX-HSU
TABLE
ET AL
I
Cardiac Catheterization
Findings October
Pressures (mm Hg) Right atrium Mean c wave ” wave Right ventricle Pulmonary artery Pulmonary “capillary” Mean I/ wave Left atrium Mean c wave II wave Left ventrrcle Aorta Left brachial artery 0, saturation Nitrous oxide from pulmonary artery Fiberoptic catheter exploration of RA and SVC lndocyanine green curves Pulmonary cineangiogram
1967
June 1971
2
3 62/i
32&/O 30-35/8-l
2 (25)
FIGURE
18-22 10 45 11518 115182
i&i
3. Chest roentgenograms
WC
May 1976
The American
1 I’;,; 6 115180
Negative for shunts Varix right Inferior pulmonary vein Marked mitral regurgitation
RA = right atrium;
22 36 24 lOOl22 100/55 (80)
3% (normal) Negative
Marked paravalvular regurgitation
= superior vena cava
(posteroanterior
and lateral) of June 13. 1971 reveal an increase
Journal of CARDIOLOGY
Volume
in heart and varix size since 1967
quired heart disease. Six patients had concomitant mitral rheumatic heart disease. In one, the size of the varix decreased after successful treatment of congestive heart failure.” TwogJo of the six patients underwent surgery for mitral valve disease. Mitral commissurotomy was performed in one patient9 whose varix had not enlarged in 5 years; 4 years postoperatively the radiographic appearance of the varix was unchanged. Prosthetic mitral valve replacement was performed in the other,lO whose varix had progres-
cases. In one” of the latter two, it communicated with a bronchus as a result of erosion into the varix by an adjacent tuberculous process, and recurrent hemoptysis led to death. In the other, the varix contained thrombi thought to be the source of cerebral emboli that resulted in hemiplegia and death.” In all four patients the diagnosis of pulmonary varix was not made until postmortem examination. In more than half of the reported cases, the varix was present in the absence of either congenital or ac-
930
(46)
1972
14-18 20-25
LV cineangiogram
LV = left ventricle;
62/32
February
37
PULMONARY VARIX-HSU
FIGURE
4. Chest roentgenograms
(posteroanterior
and lateral) of May 20. 1974. The pulmonary
sively enlarged for 7 years, 2 years postoperatively the varix was no longer visible radiographically.
ET AL.
varix is no longer visible.
pulmonary varix after mitral valve replacement in the patient described by Hipona and JamshidiiO and in our patient supports the recommendation of operative treatment in this group of patients with pulmonary varix.
Treatment The limited clinical experience with pulmonary varix precludes definitive therapeutic recommendation, but treatment does not appear to be indicated in asymptomatic young healthy adults once the diagnosis is firmly established.‘l Serial observations, including chest roentgenograms, to determine stability of the lesion may be prudent in this group. Nevertheless, it bears emphasis that onels of the two patients who died suddenly from spontaneous rupture of the varix into the pleural cavity was a 30 year old woman without cardiac or pulmonary disease whose varix size was unchanged radiographically for 12 years. In symptomatic persons with pulmonary venous hypertension due to mitral valve disease, replacement of the diseased valve by prosthesis should be given consideration, especially if the varix has progressively enlarged. Roentgenographic disappearance of the
Addendum Since completion of this manuscript, a brief case report by Perrott and Shinzl has appeared, describing a 33 year old woman with rheumatic mitral insufficiency and a pulmonary varix of the right inferior pulmonary vein. The varix was visualized at the time of “valvular surgery,” but the subsequent course was not stated. Acknowledgment our appreciation to Drs. Michael Paszek and Wilkenfeld for their assistance in the preparation of
We express
Jack
the manuscript typing.
and to Mrs.
Marlyn
Baran
for her patient
References 1. Puchelt: quoted by Gimes R, Horvath F: Uber die Varikositat de Pulmonaivene. Fortschr Rontgenstr 89:545-548. 1958 eines Lungenvarix. Verhandlungen 2. Hedinger E: Demonstration der Deutschen Gesellschaft fur Pathologie 11:303-308, 1907
7.
Hauwerk D: Lungenvarix und Haemoptose. Munchener Med Wochenschrift 70: 1084-1086, 1923 4. Klinck GH Jr, Hunt HD: Pulmonary varix with spontaneous rupture and death: report of a case. Arch Pathol 15:227-237, 1933 5. Neiman BH: Varix of pulmonary vein. Am J Roentgen01 32: 608-612, 1934 P: Phlebektasie im Lungenparenchym (tin Beitrag zu 6. Jacchia
3.
8.
9.
IO.
May 1976
den isolierten Rundschatten in der Lunge). Acta Radio1 17:7487, 1936 Case records of the Massachusetts General Hospital, N Engl J Med 245~575-581, 1951 Mouquin M, Hebrard H, Damasio R, et at: Varice du poumon diagnostiqub par I’ angiocardiographie. Bull Mem Sot Med Hop Paris 67:1091-1094, 1951 Gottesman L, Weinstein A: Varicosities of the pulmonary veins: Case report and survey of the literature. Dis Chest 35:322-327, 1959 Hipona FA, Jamshidi A: Observation on the natural history of
The American
Journal
of CARDIOLOGY
Volume
37
931
PULMONARY
varicosity 11. 12. 13. 14.
15. 16.
932
VARIX-HSU
ET AL
of pulmonary
veins. Circulation
35:471-475.
927-935, 197 1 Meszaros W: Lung changes in left heart failure. Circulation 47: 859-871, 1973 18. Papamichael E, lkkas D, Alkalais K, et al: Pulmonary varicosity associated with other congenital abnormalities. Chest 62: 107-109, 1972 19. Davia JE, Golden MS, Price HL, et al: Pulmonary varix. A diagnostic pitfall. Circulation 49:lOi l-1012, 1974 20. Bryk D, Levin EJ: Pulmonary varicosity. Radiology 85:835-837, 1965 21. Perrott WW, Shin MS: Pulmonary varix. J Thorac Cardiovasc Surg 68:318-320, 1974
1967
17.
Nelson WP, Hall RJ, Garcia E: Varicosi& of the pulmonary veins simulating arteriovenous fistula. JAMA 195:13-17, 1966 Poller S, Wholey MH: Pulmonary varix: evaluation by selective pulmonary angiography. Radiology 86: 1078-l 081, 1966 Bryk D: Dilated right pulmonary veins in mitral insufficiency. Chest 58:24-27. 1970 Viamonte M, Le Page JR: Pitfalls in the radiologic evaluation of mediastinal abnormalities. Radio1 Clin North Am 6:451-465, 1968 Perret L, Fortelius P: Ruptured aneurysm of the pulmonary vein. Acta Tuberc Stand 41:53-54. 1961 Bartram C, Strickland B: Pulmonary varices. Br J Radio1 44:
May 1976
The American
Journal
of CARDIOLOGY
Volume
37
